Underground Clinical Vignettes - Pathophysiology II.pdf

March 26, 2018 | Author: twinkle000 | Category: Kidney Disease, Medical Specialties, Clinical Medicine, Diseases And Disorders, Medicine
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BLACKWELL'SV I M B H U S W , MJl Ll~liversi~y of California, San Francisco, !:lxw o f 7(1(11 Series Editor, Diagnostic hdiologiss VISHAL PALL, MBBS Gorrrnmcnt Mcdiral Cr,llrge, Cha~ldig-~rh. Intlia, Clasr o f l9i)fi Series Editcrr, LJ, of Texas. Galveston, Rrsidrnt in Internal Medicine & Prrvcntir,e Medicine TAO LE,MD Uuivrrsity of Califoi-tlia, San Francisco. Class or 10116 ALFXANDER GRTMM, MD St. L o u ~ s Urliversity Schuol of Meclicirle. CIms nf 1999 Blackwell Science CONTRIBUTORS Robert Nason I'nivrrsit~ of'Texas Medical Rmnct~, Class of " ( 1 0 9 Tishiha Wang University of Texas M~diral Branch. Class of 21702 Kristen Lem Mygdal, iMD Ur~ivetsitvo ~ b n s School : ~ ~ of ILZerlicinr. R r s i r l e ~ in ~t Rarliolog Fadi Abu Shahin. MTI Uni~crsin, o f Uaniascl~s. Svria. Class of IY!Cl Jose M. Kerro, MD La SSae University3Mexico Citv Vipal Soni, MD LIC.L4 Srhonl nf hledi~irlr. Class nF 191)tI Hoang Nguyen, MD, MEA For tl~weqternUniversity. Clay\ of 2001 Editorial Officcs: Chmmerce Placc. 330 Main Strrct. M:klrle~~. Mass;~clii~setts ( P ! 14H. I!S#l nsnev Mead, Dxfnrrl OX? OEL.. Enalaiid 25Jul111S~r,ccl, Lo~lrluirWC1 N 'll3S. End;ultl 23 Ainslic Plscc. Lclinbul.gIl El43 641. Scntland 54 Clniv~*rsir.y Strcct. C:a~.lron. Vic1nl.i~3053. ?\listralia .%cq~~isitio~~s: Laura Uell'ortn~ Ilevelopmcnt: Amr: Nritth1-clck Pmdurtion: Imma Hind ;lnd Sliai+'tiC~i~skr-rger Mar~ii~dcuirin#: Lis:~ Fl;ula~an fiarlirlins Manap-r: K a l h l ~ e Mulcally i~ Crwer desig11by L d i c 1laimcr Inwr,ior dccig~ihv Shawn Girsberger Tvpcxet I)\:TcchDookf Printed a n d hn~lnd hv Capital City Prevv Other Editorid O f f j c ~ ~ : 1llack~~-tl W~c~cnccl~aItq-V Chn ~ ~ hl rl~ 1,g l<ui.K~rstendamln 57. 107117 H r r l i ~Germaiiv ~, Rlarkwrll Sriencr K l i hlC: l i o d t . n n ~ ~ r hRuilding. o 7-10 K O ~ C I I I I I ; INil~u~~j!xl>l~i, ~~II> ( Jli~to-ktt3 7irL.m 104,1ap:3n Irlwa S k ~ t Llnivct>~lv c PI.CLS. . I U l dh ~~ f ' ~ S 11 C~C~ICC C:urnlxwv. 2121 5 . Statr Avcnue. Xmcs, low 500 I +H'404la Ll\ i Dish-ibutors: T f l. ~ ' I ?ll<-ni<fi Rt,irk\+rll PrlhlisI~in~ c / o nrnc: P,O. Rnx 20 50 Wuizcr Spnr t Lane Willis~rm. \ T0 5 4 9 ~ 4 0 2 0 ('l*clepll<>nu nr-ders SO(Y21li-25!!2: AX o r d c ~ H ~O: ? 4 f i I-7fi26) rlzirfrrrlin Blackwell's Underqround Clinical Vifpettes: Pathophysiology TI, 3e ISDM O-(j39-04.55?-1 'l'he BlacliweIl Scicnre lngc i q uadr mark of RlarkweII Sc irncr Lirl., i,c.giutc.rcrlAT thc L111irerl Rrngcloni Trdcle Ll.la~-LqRcgi5irv T~I,IL~IWII Scivl~cc I*!\,, l,ic!. 54 Ilnivcrqiry Strvet IL~rliun. l'ictr>ria :%f15:% (Trlrpl~oncnrrlrn: OL%934'i-0300: tLm~ ~ r r l03-~~3~1~4-301 e~~. ti \ OTI/$II!P '/'I).+ A I ~ I ~ ~ I I(trt11 YI,A I ~I+/TII/~~< Rl;tcknmc~ll S( ieilcc. L.tcl. C / O Mllmlnr~ Bonk k n i c r c . I d . P.O. Box 2li9 Library nf Cong-ess Catalo~ng-in-Publication Data Rhushan, Vikau. BlacLwcEl'c iulrlcrgrout~d c l ~ n ~ cvim-nptre%. al I'a~l~oplivsiuio~ J aulhor, t'ikac R l ~ ~ ~ r h an 3rd . ed. p. : cln. - ~ L ' I I ~ I c ~ , ~clinical cIII~c \ iI~ 3 e r r f i j KPV.ed. nf. Pa~hr>phvsiol~~gv / \Skh< D1111~ll;ln. 2t1rl crl. ~19'39. ISRV Ofi324)45:>I-.5 (phk.) I. Pl~!?iinlr>gy, Pat11rhr)~ral - U s e str~dics ' 1 . Ph!ulcran~ - L.iccnses - Lln~ter! Stares Lx;~n~inatlons - Studv p i d r s [DNI ,121I . Clinical Medicine - Cntse Rcport. 2. C1iiiic;~l hledicint - t*tnl)len~s and F:Xer(.i~e~. WR 1 H . 2 R37.ihh 2002J I Tide: L ~ n ~ l ~ r ~ clinical ~~~tricI \-i?pieuc. Pa~E>oplly~ir,lugy 11. I*;ill~oplivsinlo~ II1. Iitle. 1Y. bcrlcs. RR 1 13 R45D 2002 lilG.07'07tLdc21 hbing<lon Ounn O X I 4 41'K ErigLlnrl (Trlephnne nrc1lt.m: 4-W 123T4fi500: I A K ori1c.n: 4-P 11 2354fi5r755) All l i ~ h t res~ivctl. s Kr, ]la!-Iof this honk m:w h rt'pnx1u~~cl in itlit. f r ~ m vr Ilv ;~riy rlcctn,i~ico r nierlianical means, incl~lrling infomiation storage ;unrl rc1ricv;)l svslctns, u'~tllr>ut pc~,n~iscimn in writing frnln thr pt~hlislirr.rxrrpl hy ;I rrvie~vrr who may q l ~ n t I,littf r p&svaRes in ;t revit-w. ~nct-s. ~thy t tinic. . of pul.nLcliW ~ L ~ l l l li > s c ~ ):ht~cl k clisclairn any linhiliry.1~ acrlwate F r ~ all r ~irci~nlsr. d a m a ~ r inc~~rrrcl a . :.mrl a ~ i t l i n m dn n o t g~lk1miI~C [he ' ct.No tire The a ~ ~ t h nof' i s this volume have takrn care that rhr infnrmation ct~ntainrdhr.1-eir~ is nccunte and cornpatiM e with rhe swncl:~rds genrr.r .>I' IIIPus? anrl appliration r)i' anv n i the ctmtents uf ?Ibis\.ic-r-epred . ~ I C . i t is ditficult to enrurc that :ill rhr infnrniaeioll p+n is rnrirt. 01. .ol~irnc. direcrty u r irtdirert I?. Ins?.11~.licatiort.puh1ishc. Nwcrrl~rles~. a consequence. -' Anemia-Autoimmune Hemolytic 1 Antiphospholipid Antibody Syndrome !' ' Burkitt's Lymphoma 1 Chronic Lymphocytic Leukemia (CLL) I ^' Chronic Myelogenous Leukemia (CML) ': .CONTENTS Acknowledgments Preface to the 3rd Edition How t o Use This Book AbbreGations General Surgery x xiii mFi xvii Abdominal Aoxtic Aneurysm Acute Cholecystitis Appendicitis Cecal Carcinoma Esophageal Carcinoma Gastric Carcinoma Gastric Leiomyoma Intestinal Obstruction-Acute Tntussusception Pancreatic Carcinoma Down's Syndrome Marfan's Syndrome Acute Lymphocytic Leukemia (ALL) Acute Myelogenous Leukemia (AML) B Y I= r . s ! Disease Imfi Histiocytosis X-Letterer-Siwe Disease 1 Aadglcin's Lymphoma " 1 Idiopathic Thrombocytopenic Purpura (PP) ' 1 " K C A Multiple Myeloma Myelofibrosis with Myeloid Metaplasia Non-Hodgkin's Lymphoma Polycythemia Veza (PCV) Sarcoma Botryoides 7 ' 'i A - I i I: . .."' " I Anemia-Aplastic .7 Deep Venous Thrombosis Disseminated Intmvasmlar Coagulation (DIC) ' ' ' "I Dysbetalipoproteinemia Hairy Cell Leukemia Henoch-Schonlein Purpura Histiocytosis X-Eosinophilic Granuloma Histiocytosis X-Hand-Schu12eer-Christian P * -a ""$ P .I Sickle Cell Anemia I . < I -* p. AA % '7 + -"" ? : I Genetics Hematology/Oncology . r7- 1 1 Renovascular Hypertension Seminoma Testicular Choriocarcinoma Testicular Dysgenesis Testicular Teratoma (Mixed) Testicular Torsion Urate Nephropathy Wilrns' Tumor Adult Respiratay Distress Syndrome (ARDS) .Immunology Infectious Disease Neonatology Thalassemia-Beta Thrombotic Thrombocytopenic Puxpura r Transfusion Reaction-Acute Hemolytic T m s h i o n Readon-Febrile Nonhernolytic F Von Willebrand's Disease . " (MPGN) Membranous Glomerulonephritis Minimal Change Disease Prostate Carcinoma Renal Cell Carcinoma Renal Infarction mI . I " > i I " . Nephrapathy Diabetic Nephropathy Goodpasture's Syndrome Hypertensive Renal Disease IgA Nephropathy {Bergefs Disease) I -Lupus Nephritis Membranopro~iferativeGlomerulonephritis " > 8- rn '- 8 " ..I Waldenstriim's Macroglobulinemia " . Pulmonary Em FT I Asbestosis Asthma Atelectasis-Postapexative 1 1~ Bronchiectasis . Wiskott-Aldrich Syndrome Serum Sickness 1 * Syphilis-Tertiary (Aortitis) Erythroblastosis Fetabs Sudden Infant Death Syndrome (STDS) ' ' Acute Tubular Necrosis (Am) ' Adult Polycystic Kidney Disease (APKD) Alport's Disease Amyloidosis-Primasy Benign Prostatic Hypertrophy (BPH) 1 Bladder Cancer Bladder Outlet Obstruction. Churg-Strauss Syndxome COPD-Chronic Bronchitis COPD-Emphysema Fat Embolism Hypessensitivity Pneumonitis Idiopathic Pulmonary Fibrosis (IPF) Lung Carcinoma Malignant Mesothelioma Pleural Effusion Pneumothorax-Spontaneous Pneumothorax-Tension Primary Pulmonary Hypertension Pulmonay Embolism Saxcoidosis Silicosis . For submitting comments. Benjamin I-I. Cunningham. Rajeev Cuptit. Eric Jack~on. Dave Brinton. thc Armed Forcrs Institiare of' Pathology. DeMrllo. Lisa Flanagar~. H e n ~ y E.Thro~~gho the l ~ tproduction ofthis book. proofreading. Pat11Konity. Mona Pall. Kris Alden. '4. ICessclheim. Emmanliel rle la G ~ I zRobert . IrIadirnir Col-ic. o Kevin Leung. \. Arnold Cheung.Ronald Cowan. Scott Lee.mk: Tam Adamovich. hdolf Etchegaray. PLlldrua Fellows. Slla~vn Girsbergel. For help with securing images for the ell tire series we also thank Lee Martin. Francis Kong. G u i l l e ~ ~ n Lehmann. Lynlna~z Racolor.14. AIcx Grimm. Sanjay Rindra. Lau. Pat111 GoyaP. we have had o he supt1n1-1 or man): friends and colleag-ues.kv. .John Dabid. we c o l l ~ tii~ely c t1i. Rajni K. Sharrnila Disqanaiku. David C~nel. Tamara Callahan. Das. Ken Lin. Frase. Alex Cirimm. Dchas11isl-iRehem. Tony George. Aarchan Jnshi. Ileam Bartholomew. Warrcn S. Michelle Hig-ley. Carolyn Alexander. Kristirl Caumt-1: Yohanncs C~hreegsiallher. h m a D i ~ ~ i c l a r n ~ Sl~nir ~ d i . Hehert Chen. Sana Khan. Natalic Barleneva. Kristopherjones. For prior contributions we d ~ d n k Gianni Le Nguyrn. hlea Elwehio. Sumit Bhatia. Dan I-Ioi t. Samuel Chumg.ekjn 12. Simion Cl~iosea. ne Alexi~ndelBrnwnie. Priscilla A. Yoon Cho. Jonathan C:heng-. Sue Hall. <J~~lian Brown. h i 1 Gehi. Rryatl Casev. Lorna Hind and Gordnn Tihhitts. Juitla. Special thanks to o u r support team including Anu Gupta. Andrew Pin-wei KO. and assistance across all oS t h e vignette titles in all editions. Govancn. h r n n S. Krackov. Sonia Santos and Elizabeth Sanders. Sundar Jayarman. Faiya7 bpadi. David Hasselbacher. IYarren Levinsoti.S. Pci-Ni Jone. An11 LaCasc~. Tina Pan iz7i a i d Petcr Anderson at t h e University o t Alailama. Navncut Dhillon. Connie Lee.. David Donson. including Laura DeYoung. Srishti Gupta. Sean Dalley. L. CIlristoplirr Cosgr-ove. corrections.-Toriathan Kirsch and Chirag .ire have enjoved working with a wnrlrl-class ii~ternational pub lishing group at Blackwell Srirnce. Ahmad Halim. PaliI Levett. Seth Karp. Eric Ley. Amv Nu tthrock. Tirn Jackson. Talm Mathur. Arnold C h i n . Ryan Arrrlando Davr. and many of our fellow Blackwell Science a ~ l t l ~ o r s . Gretchen C:onant. Kar. David Frenz. Aryan. Anastasia hnclcrson. editing. b i n . Aaron Gaughey. Tamra Heimert. 5. 8.2. A n t o ~ ~Talavern. Rick RuIler. lO. 2. 18-31).20. '7. fIe11nessy TPJ. 1 9 9 7 .6.8.105b. Reatriu Reig. Berg D. Lillian Su. I.3. Eunice U1ang. P a ~ d Pamphn~s.MT5. Palance.2. J. Justin Smith.65b. Okafo Uchenna. Kcarclo Pietrohon. Figl~res 7. 2. Figures 2. 57.4. Richa \. Diego Ruiz. George Noumi. Tai Roe.7. A d-r~nnrpd Clin &.Gillespie S. Michael Murphy. Edition. 2000.27. Lecture hlutrs m r M~diral 12fiml)iulu~ 3 ' ' .9.20b. ~ M e d i r i ?Osney ~ ~ . Osuev Mead: BIackwell Science Ltrl. 10. Mark T a n a h . rve collectively thank the staff at Blackwell Science in Qxford.7fc.8. Jess Thompson.14.52. Mark Maddox. Indi Trehan. MT5. Brian Yang.gtia. 11. Rim Suti.3U. ilc391j. Jererny Richmon. John Wages. S. Aashira b n d e r i a .15. 7.10. 2. 2. Rajiv Roy. 2.16. Mead: BIackwell Science Ltd.35. Alan Wang. 5. J>esselberger U. 14. Figurc~ 12. 34.2. 18. . ~ 18.JinfiaPark. 29. Ashraf Zaman a n d David Zipf. Cawell h'gnyen. 2.a/Skillr mnd Phg~icaf L)inposi<c.tj5a.Jol111SnlIak.Pavel Lobanov. 7. 12.3. . Craig Nodnrft. .~~li/ion." I':clirimn. Mead: Rlackwell Science Ltd. Rachan Reddy.13.5. Rowley DA. 7. Isahell Schmitt. Eipi-cs 20. c ~ 2"" . . Reg): N.. Figures 2. .8. 16. Ginsberg 1 . 8. Maiilou Reyes. 14. 35. 7. 7. 18.4. Sarnir Mektit. 19. 15.rciure lVotes on Nmruluml.28a.733. Eric Taylor. Ulmimala Sal-kar.Lf. 1999. 2.18.10. 2. 8. Seth Sweetscr.-JoeMcssirla. 11.23. Siva Narapan.33.22. 18.10.tcl. 2000.6. 2. * Elliott T.'amla.John SchiIling.65c. Linh N p v e n . Gil MeImed. 17. F i ~ i r e s 2 . Daren SchuhmacI~er. Kay~noncI Turner. Barnford K M~ifimf Mirml~ioktgand I! fPction a fa Glrirrrr. * Bannisrer R. C l i n i r n l . Bostnn. h r genernuslv conrribu ting images to thc entire Uulrlw~ly~~ind Clinical I f i g x p l t p Step 1 series. Sanjap Sahgal. io Merita 'San. Eric U y p a n c o . and Bcrlin as well as: I\xfnrrl. Osnry Mead: Black~vell Science 1. Vivek Nzlndkami. 2.21. Anthong Russell. Rlnrkw~ll Science Ltd. 2.103h. 3. 1996. 1P.Julie Sundararn. h d y Mreiss. RahI. 3.50.25. Fadi Abu Shallin. Sonny Palel. J.3. Osney Mearl: BIachvell Science Lid.43. 5 ' 1 1 ? ~Iq)sney . Dcanna Nohlt-za.16.1. Ruberr Mows. Okrida.3:!.36.#lh.2%. Aram Mardian. C~~srhiel-i A.9. Rim L.13. F i g ~ i r e 13. 27. Soi~aI Shah. 8. Hastingq M. Greenhalgh RRM. 9.26. 1999.31. 2. Hatry Zaky. D a r i t ~ T. 7.2.27. 11.19. I.28. 7. * Gitlespie SH . Osiley Mead: Blackwell Sciet~ce IAd. Mae SheikhAli. I.3.5.%my Williams. Edic Shen.38.%darnI. 12..12. 2. Grace FA. 5. 5. 8. Inf~ctiuusI l i s ~ c t .6. 2. 22. Hornrand Ak! Hn~rnnloln~v nl rt Glrmr~. 22.3.1. F i p r e s 22. 2000. . a n d we will br l'leaw let tis know if' your name has been rnissecl or misspelled happy to make the update in the next edition.Mehta MA. 00~ney Mcad: DIackwc11 Science Ltd.2. Each atlas image is descriptirelv cagtionrd arid linked to its corresponding Step 1 case. divided into gross pathology. Over the last hvo years mrc have accu~nulated and incorporated over a thousand "updates" and improvements suggested by you. the third rdition sets now include two brand new COLOR ATLAS. \Tell nver 1 00. including patirnt images.000 copies of the LTCLT book4 are in print and have been used bv shldcnts all over the world. and mictol->iology ((smears). . hematolop.vhelrningly positive stliden t reeclhack Tor the 2nd edition of ollr I h d w u n d C?inirrc! ~ ~ i b r l l ~ / ~ f c series. Step 2 case. includulg: I I I * many additions of specific boards a i d wards testable contetlt d~lerinns of reclundant and overlapping cases reordering and reorganization of all cases in both a new maqter index by case name in each Atlas correczion of a Cew Cactrial errors series diagnosis and kcarmen t updates I * addition of5-20 A nalnm? new cases in every hook * and thr addiriorl of clinical e x a m photographs within LTCb- And most important of all. supplcments. dermaloIogy and hmdus- copy. * The CTCIJ--13ccsirS r i w r p Cdur Atlar I ) includes over 250 color plale5.eaclers.PREFACE '60 THE 3RQ EDITION Wc were v e r y pleased with t h e ove~~. and /or Step 2 MiniCasc. our r. one for each Clinical Vignette serics. mici~oscopicpathology (I~istology) . (St* Thc I 'CV-CJdni(*rrlS r i m r ~ C h r Atlas (St+ 2) has over 125 color plates. logv O R = OB/C. P2 = P a t l i o p h v s i o l o ~Vnl. 03.~f ilidjnr thr! ITTiVILl St+ 2 (12ppleton kk Lange/McCraw-IIill) honk will be linked to the corresponding UCV rase. Vd. etr. D indicale up to 4 ima~es.popular First Aid fw thr i:SMI. if'rhe Case numhcr (032. If~helitlkisnotbold-faced rhi~ indicates that thelink is inclirect (Step 1 c x e wit11 Clinical Sciencc Step 2 Atlas Iink u r vice versa).How Atlas Links Work: Step 1 Book Codes are: Step 2 Book Codes are: h = Anatomv RS = Beha\+c>r:tl Sricncu BC: = Rinrhemist. ER-0350 /M-p3-032A Image: I [ = f lumatnlnp PC.ange/McGraru-Hill) atlrl Irir. I I M ! ' = Internal hlpriiriue.\N PED = Pe~liatr~cr SITR = S I I I X ~ ~ ! PSY = P~yrli~atry M(: = ViniCase ER-035A. 111 PH P l ~ ~ ~ r ~ ~ i ~ ~ c o l o ~ ~ ~ . then thcre is only cine image. Vnl. * IT the link i s l3old-faced this indicates thal the Iink is clirect (i.7 5 4 1 = J~!irrc>hinlt>~. . ~ 11 P. we have cliosen tn cross link he Slep 1 and 2 series.7 = Pa~huphy>ioIuby. I ER = En~ergenry Medicine I M l = l n r w nal Merliril~c. \bI.ui~. (:rrlss UCVl or UCV2 Series Pathnli~v PM Microcctrpir lbarIiol[>p . Otherwise A.l~iology Vnt. i & havc also irnpl~tn~nred a fm structutal changes z~pon your rcqucst: Each currcnt and fi~riire edition of mu.. 11 hEL1 = Nt. C. n l o I~ .e. .IS Strj) I (Appletoil Pc T.) is not f o l l o ~ ~ e by d a letter. as thc firsf A it! t>ooksare re\riscd yearly. IT P1 = P a t l ~ n ~ ~ t ~ y s iI'nl. \& eli~ninateclUCZ' + Fimt Aicl links i u they frequently hccu~r~e rjltl of date. Indicates Type u f \ n Case Number Indicates . 1 Mr?= hiirra. Bold Faced Zinks: Irl order to give you access to the largest numbcr of-images possiblc. Vfh. Step I Case with the Basic Science Step 1 Atlas link).5. B. please also e-mail us your CW/resurne. usc the follo~vingrllailing address: Rlack~vell Publishing. and hrlpful For both licensing exams and the warrls. Attn: UCT Editors. additions o r ncw l-ignet~es we will also consider inviting you to become a "Contihutnr" on the book o f your choice.chon Vishnl Pall Ton I. USA. Viltos Rhws/. U'e prefer that you submit correclions o r sugg-cstionsvia eIectronic maiI to UCVteam@yahno. Midden. 350 Main Str eel. MA 021 48.vely) to read.eloping additional books. Kyou do not have accesq to e-mail. They are designecl to he easy and ft~n (cornparat. Fnr the f i n t subillission of tach factual correction or new vignette that is selec~ecffur i~~clusion in the fourth cdition. If you are interrsterl in I~ecoming a potential "Contributor" or "Aulhor" on a future LTCV book.P Octob~r 2001 . or-working with our tear11 in cIe~. If y o u snbmir over 20 high-qllality corrections.com.* The Color Atlas is also specially designed for ql~izringcaptions are descvi ptivp and do not ~ i away ~ e the case name directly.FIcase includr "Underground Vignetres" as the subject of yol~r message. We hope the updated GCV series mill remain a ilrliql~e ancl wellintegrated st~idv tool that provides compact clinical col-relations to basic science information. We invite your corrections and suggestions Tor thc fourth edition of these books. you will receive a personal acknowledgernenr in thr rerised book. $/PSI I (hppleron 8: Langc/McGmw Hill) and fin! Aid . ~ A P S )in parent tieqeq. bc considered as I a u r solr wurce of information. Other pal-en thetical rcrnark~ oftcn ~-efcr to thr pathophysiulop or mechanism of diwaqe.I. The ~ i g n e t t e s represenr com~msite\ drawn from general and qpwialry testhook?. Cases are not meant to simulate a potential real patient or an exam iignctte. tJw tCrSiIfESi+ 2 {Appleton II: Lange/McGraw Hill). The forinat shot~ld also help stl~rlerlt? learn ta present cares ~ i ~ c c i n c tduring ly oral "huller" presentations on clinical rotations. including the LTSMLE Step 1 and Step 2. T h e information pro\-icled in this hook has 13een prcpared wit11 a great cleat of thought and careful rr:search.15 a crmdcrlsrd review.iar-ily expected to he fol~nd in any one patient with the rliseasc. reference honks. tFlousands of USMLE swle q ~ t e ~ t i o n and s the personal experience of the authors and r~viewers. . Comctions.: . placed within the vignrties in (SL~AI. All the boldfaced hbbuzzwords" are for learning purposes and are not r~ece.fm. 'ach Althc~ugh each case tends lo present a11 t h signs. ho~vever.. patients genera 1 T y will not present with such a "complete"picture either clinically or on a medical examination. sr~ggrs~ions and sithn~issions of new cases are encoi~ragecl and will I>eacknowlcdgcrl and incorporatrd when apprnpriate in fit ruse edE [ions.. LTCV 1 book uses a serqies of approxirnatelv 100 %upraprototypical" cases a q a way to condense testable f a c and ~ associations. Thc cases are mcant to serve . ancl cliagnostic findings for a particulzr illncss. Definitions o f scIrc tud important trrms arc.Tlris qeries was originally dcveloperl IO adcll-eqs the inc~exqinji nt~mher c~f clirlical vigmecte ql~estionq otl n~eclical examinations. It is alsn designcrl to st~pplement ant1 rr~rnplemen I the popular ATTI Aid /.7r I ~ P CL%iZJ. Tllc clini. not as a prirnarv reference. This br~ok shoulcl not.cal vignetres in this serics arc designed to iricorparatt as manv tcsmhlc facts as possible into a cohesive ant1 metnorable clinical picutre. ~ symptoms. 1.5-ASA -4BGs hRVn . CML CrnT CNS COPD CPK CSF CT CV.rcin/b~eomycin/vi~icri~tine/dacarb-nxine angiotunsin-convertin g en q r n e adrenocol-ticotropic hormone aritidt 111-eticlinrmone alpha fetal protein aortic insuff~cierlcy acquired iinm~~nndeliciencv s~ldrr)me acute lymphocytic leukemia nl.ICE ArnH XDF-I AFr' A T AIDS ALL ALT AMI.~nine t ratlsaminasc acute rnyrlogenous leitkrmia antiiluclear antibody adult respiratory distress syndrome atrial rrpml defect ail ti-ctreprolysin 0 aspartate transaminase arteriovennus h-niiiam enema hlnod preswre blood rtrea nnlrogett coronal? artery discase comnlon acute lyrnphobla5tic leukemia antigen completc blood count congeu~ive heart failurc CI-eatine kill as^ cElronic 1)qnphocytic leukemia chronic myelogennlis Ieukemia C)'~OJI egalo\7irus ~ central nen70ussyiterrl chrnnic nb~tr-ucrive pulmonary disease ci~atine phosphokinase cerebrospinal fluid computed romogl-aphy cerebrovascular accidenr chcst x-ray clisscminaied itlrl-a\mcularcoapibtion dislal inte~=phalan~eal diabetic kctoacidosis diahetec mellitus deep tendon reflexes clcep venous thl-onlhoqis .K h m s ASD 'IS0 AST iZV BE BP 13UN CAD CALLrZ cnc CI-IF CK CI. 1l.4 rnR nIC DIP T)KA DM DTRs DVT 5-aminosalicylir acid arterial hlood gases ad tiarn. P T .yt PS mt-an corp~~rcular Il~moglohin concen t r ~ ~ t i o n MCHC mean carp~~scnlar volurnc \4c\nlrrltiple e~~rlocrinc neoplasia MEh- .e kidnevs/ttretrr/hradclcr KLrR lac^ ate dehydrogenase LDH lower esophageal sphincter LES lilrer.i n~ellitm IDDM imrnunoglohiilin Ig insulin-like g r o ~ q h Ijctor IGF itn tmtnuscular IM jugular venous preswu.fi~nction tests k. I-IEENT head.F-s% lumbar punutu7-e LY lcft vuntric~~lar LV left ven i r i c ~ar.~~c~iu~aphy cjrction fwctior~ esol>haqogaqr mds~nrlenoscopv electromyograpl~~ encloscopic rctro<qaracle cliola1l~opalzrreat~)grap1~~ erythrocyte sedimentation rdtf ESR fi~rcrd cxpiriitnry vnl~tine FE\ ' fine needle aspiration FNiZ arllokody absorption FTIZ-A RS fluoscszcnt truponrmi~l f{~rcecl ti tal capaoi tv FVC glomrnllal.t r o p h y LvH electrolytes T.pher<)c~locis I IS idenrification and chief complairi~ ID/(:C: insulin-deperidm~ dia11e1e. now. ears.. eyes.tor ge~iiiot~~ii~a?y GU hepatitis A \ims Hrn' hr~maiz chorionic go~~adotrc~ph in hcC.:\ history of pl-esen ill ~zess 1-1r l hear1 t a t e HR Imrman l-ahies imrnunc globulin HRrG herrdi tam . ~ l1 1 ~ ~ ~ 1 . and thl-nat human i r r i mruiorleficiencr.Siltralion 1-ate GFR !growth hnl-monc GH gastrointestinal GI GM-CSF granulocyte macrnphagr colon? ~tin~nlatinfi f ar .. vin-us HnT 11u1nanleukocyte antigc11 HI.ERlJ ECG Echo EF EGD EMG ERCP Epstcin-Barr vir-us rlcctrocardiograpFi~ rchuc. A LiGI ul-innlysis upper GI rs .inc~ist ine (Onco\wrin)/ procarhazir~e/pi~prl~~ison~ rnagn'ctic rrsoilance (imaging) IIO~I-Hodgkin's lymphoma non-i1isr1lin-rle1~~11clent dialrrctcs n~ellii 11s nil per os (nt~thing Fly rrlou th) PIP PRS PE proximal in terghalangcal pt~ysical csam pulmorl. F I ~~tv-th I rc111g11 wndrorne of inappropriate \ecrriic~noTAD1-l scxuall!r tranlirni~ terl disease ~hyroirlr ~ ~ n c t i o tests n lli~roid+timt 11aiing Ilorrnonc total iron-Frinding rnpaciry liansiug~~lar intrahepatic portosY4stenl ir s111~1a t STD T I T 5 1P'l TSI-I TIBC TIPS TPO TSH Trl' LT.uv Funrtinn P ~ s PM I I'M N PT PTTA PTH PI-I' PL'D RBC RPR RK lerts RS RSI RVH SUFT STAIIH S LE ~~~wdivvm lzorillonc irl partial ~hromhoplastiri ~ir~re prptic utccr disrase red blood crH rapid plasma l-eagin respiratoq mtr Rcrd-Stn-r~herg(cell) r.chairiii~eJ\.MGUS MR KHL KIDDM NPO NSXl D PA mvocardial infarctio~i mcchlorc.e~itrir~~lar rigl~t v~ntricular hvpertrt~plir mall 1 x 1 ~ el .i~l>\ t . ~rdia wllite blond culI 1's IT M'BC M-PMr SR Il. Vcncrcal 13iqease Research Lal>oratory vital s i g n s ventricular tac11vc.blff-Parkit~son-'It'tiite(s~di*ome) x-ray .VIIRI. Sequelae incluclc rupture. eccentric m ~ ~ r a l thmmhus wen. CTJUS. Surgical replacement with graft {if > 5 cm or symptorrla~ic) . large. hrlenrysm wall contains all three la!.g. syphilis). cystic medial degeneration. P ~ T ~PG-P2-001 T-I Atlas Link A B D O M I N A L AORTIC ANEURYSM . 2 m m s C V. artery disease. painless upper abdominal mass approximately 3 cm in diameter.af't.urctcrs. a n d compression of acljacen t structures ( e .et-q (inritna. g .catiorb: thron~bus may alsn he 111-esent: in trnrnural clissectinn nlay also he wen. and art eri tiq.O C C I L ~ S ~ O ~ ~ secondxr-y ro rhr-mnhus fortnation. Discussion The iisk nf rupturt*with potcntiallv fatal bleeding increases w i t h sizc. vertebrae). . laletxi: calcification of aneurysm wall. I t is also rn11scd by trarima. media. embolisalion.. abdomen: dilated aorta w i l t 1 il-regular calcifierl wall. D G) m . V~SCIIIJI. i~~feclioxi (r. HPE T h e patient has a history of occasional abdominal pain and hypercholesteroEemia that has been poorly con tl-ollerl hy diet ancl mr-dication. Imaging . Al~dominal aortic aneurysm i q ur~rallv cauwrl by atherosclerotic rlispase and is often associated with coronar?. KUl3. infrrtioi~. D < Gross Pathology Mosl aneur>'srnsaye Incated Iwtweet~renal arteries and iliac hifi~r. PE Pulsating. Micro Pathology Treatment consider endovascular sten t/gr.IDJCC An a~y~iptomaLic 60-year-old white male undergoing a routine phyical exam i s dismvered to have a prilsating abdominal mass. advent i tia) (''IHI~F'' b \ ~ ~ ~ ~ ~ % h ~ ) . eral pi. Clinical risk factor-s inclttde tl~c four F s :fat. tachycnrdia. Imaging Cross Pathology Gailhlaclrlcr inflammatio~iranging tioin wall ecleini~ to acute gang?-rncI\+ th ~iccrr~sis. analgesic$.rntial rliagnosis i n c l ~ ~ r lappcndicilis. ancl per. Elc\r~ted clii-ec.1bilir~rl~in . Shc also complaii~s of' nausea anrl has vomited thrce times. female. # j ~ l l l ~ l . ~n Micro Pathology Treatment Conscrvarivc trc:ltmcnr includcs n o oral i~ltakc. c. DiiTei.veIol>sacute intermittent pain in the right IIpper quadrant ant1 r i ~ h scap111a t after eating a fatty meal. dt. hypoilctiv~ lmwrl sounds.{l ly li~paroucol~ic) is rlclinitivc 1i.ratmeitt. S I ~ I I P IXI-P cotnpnwrl of cholesterol: less commc~n are pigmented stones made principally ol. elevated a l k a l i phospl~atase. forty.~~e< ~ l ~ l ~ r -C ~ Jir S rit~rr ~ nhstl-uction by stone. TCT fluids. pur fo~-m.HIDA: hilure to \<stla!i7t.CRC: Ie~~kocytosis with mild neutrophilia. Discussion Atlas Link * ACUTE CHOLECYSTITIS .i l peptic ~ ~ ~ l c r~iv~!c)n~pFiri t.ltion with p ~ r i i o ~ i i l iMOSI s.fnr. PE Labs Hvpel-rliolc~terolenlia. rli~sogastric aspiralicyn. Skrr has I~ikcIsr\. and riqht lower lohc pneumonia.1rncnuj11~ a i e r I>ilir\~hin l and r a l c i ~ ~ salts.I DJCC h 42-year-old wI~itc female..il~. HPI She is nCNative American ancestry anrl i. ~epsis a~ld i l l I ~ pmlpal-turn P qtate. NIK.ini. tiq. ~>~i-fi)~ t-d .~t irlr~.~ panci-catitis. PE: obese. epi~orles ol \ i ~ n i l a r pain rolluwing mrals. srvcrc traunia ~ 3 1b u r i ~ smid . Althou~h cal~iili arr involsccl in moqt c a w s oi'actile rhc~lecsq~itis. ~itidic-. and fertile. ancl anlibiotics: cholecystectomy ( I r. llic mother of five. m\~orardial infarction. 30 pn~uncis overweight. IlS: rIistcnrled gallbladder with d thickening r o r ~ t i ~ i r l i i ~ g mliltiple echogenic shadows (stnncs). VS: I'cvcr. acaIculous cases aricr a f1e1ni tnhiliat y inainr surgeries. tcnclcrnrss in l i ~t upper l ~ abtlomiiial rl~iarlc~!i L w i i 11 inspiratory arrest on palpation (Xlu K I ~ II ~ SH S A ) . CI rn PE 1 ' s : mild tarhvrarrlia. I!A: t~ol-mal.R~~IZN~. The p ~ a k incidence or appendicitis i s in tlnc second ancl bird decndes.~ql~r Fecalith i n rig111 lowrr qr~adrant. an-a2-003 APPENDICITIS . pain on o-vc~lation ( ~ ~ r r r .'I"S POIV 1. Imaging KLJB: right psoas siradow hl~11-red: ~ent-ralized ileus with air-fluid Ie\. rnesent~ric lym[rharlrtliris. HPI Four hours after 131-eser~htion. rig11t IOIVP~ qltadranl pain elicited 1 3 ~ passive hip flexion (PSC~AS S I . >K Z m m Ln ? 5 m G) 30 < Labs CRC: elevated WBC counrf predominance of neutrophils Normal serllrn amvlase.ID/CC A 17-vea~old tnale snident presents with anorexia and poorly Ioci~liretl ge~umhilical pain followed by nausea and two cpisodcs of vomiting. lotv-grade Cever. the pain shifted ta the right lower quadrant and lnc devcIopcd a low-grade fever. Gross %thology Treatment Discussion AtZas Links ITElTII PI-a2-003. Differential diagnosis should inclt~de rnrel-oculitiq. late lecion: purnlent ex11 date wit11 ilccrosis and pcsforatioit. and Crohn 's discasc. Appendectomy with preoperative antihiotic coverage. Meckel'r. pain localized to junction of outer and middle third of the line from anierinr snperior iliac spine to l~mbilicus (M(. increased sofl tis~lle density in sight lower quadrant. ~ s r : ~ arrd -~~~uzj. L T S : noncompressible tubular strl~cturein right lower quadrant. per-i~oni tis.1 .acute salpingi~s. &rlv Iesinn : hyperr~nic appendix with Iihl-innus exudate. and gunm~lizecl or wo~rncl srpsis.N) ~ atid bv pas~ive intcr~lal arid external rotatinil of hip ( ~ ~ ~ T L * R A T C sicw). Complications includc perforation. para~i tes. tli\le~. forcign bodies. pain in right lower q~mclrartt when pressure applied to left lower qr~adran t (Rol-srnn'q SIGN) .lict~litis. ectopic pregnancy. PE: right lower quadrant tenderness with guarding and rebound.els. periappendiceal abscess. smalI rarIinp. Caures include obstruction by fecaliths (33%) ancl lymphoid hyperplasha (GO<% "u) it is occasioni~H): cazisecl kv ttlrnmrq (carrirlnitl turnor i~ l l ~ e inns1 conimc)n Lllrnor of the appenclix). fecalizh occasionally pi-esunt. O O ~ 9 CECAL C A R C I N O M A . Gross Pathology Micro Pathology Treatment Righr hpmicolec~nmy w<th remporary colostomv.~rrinoma i. US: metastatic hepatic nodules.i I ~ screening v l'nr occult blood in the stool.t-1%. Fungating. ~ e s ~ i ln e~ .P ~ . palprrhle I r f t ~111~1-aclatic11lar lymph node (VIRC'I IOU' 5 NC)I>F) : palpal>le niaqs in right iliac fosqa: hepatomcgaly. adjuvant c h ~ ~ nh~t-apy: c ~ t ToSlow up for recurruncc by mori itori ng C:FA levcls. ~ n s t r i c t i n ~ . It is the suco~ld most conlmon rallse or umnccr d r a t h . Mar-kerl pallor. i t s ir~cidellceirlcwaseq markedly alter age 50. Discussion Atlas Link mP G . Cauliflower-like.HPI She 11x5 atso notired sipificant weight loss and intermittent diarrhea. ~ t i c Positive stool guaiac test: rlrvatcrd serum rarcinoen1l9r~~orlic . nonobstnacting growth in cecum: Inav hc polvpc3ir1. mass in cerum.t tigen (CEA) 1ez. WellLrliiTerentiated artenoc:~rcinoma. h ~ p u c h r ~ ~anemia.i r r q ~ ~ l ah~ngaritlg i. Imaging RE: Iai-~e. ~ t . Colonoscopv: largr fungating growth in cecum. PE Labs C:RC/PRS: microcvtir. Earlv detecuon of cccal c. c. . pale C l rn z m 7u PE ! e . PG-P2-OOSA.tl esophagL~s is causcd hy chronic. oclynophagia. Laser ablatiori of t unmr with palliative slenL placement. . palliative radiotherapy: surgical rrsecliol~rollowed by cl~emotherapy plus radiotherapy for curable tumors. progressive dysphagia. A less cnmmoiz mriant is adenocminoma.ID/CC A 6Ryear-olcl hlack male pruse~lt~j ivi lh anorexia. Gross Pathology Micro Pathology Treatment Discussion T h e most cornmu11 variant of esophageal carcinoma is squarnous ceU carcinoma. Imaging UGl:irre@arfungaTatingesophagealmassinmiddletl~ll-rlof nhstt-~~ction. chest: ii-regular eqophagus ui th esophageal m a s with invasion of mcdiastitilrm and enlarged para-aortic lymph nocle. Emxciadon: fixecl. and regurgitation: and scvcrc dysphagia with solids. hyoalhuminrl nia. CT. and is more coinnlon in whiles with Barrett's (glandlilar-metaplasia of thu squanluus epi theli~irl~ of thc disl. arlrl weight loss. nonpainful supradavicdar node. Large fungatirig mass prorruding towdrrl esophageal lumen. Sqnamot~s cell carcinoma on biopsy. PG-P2-005B At\as Links * ESOPHAGEAL CARCINOMA . His history also rcvuals heaby alcohol intake. evenl~ral gastl-ostomy tuhr pl~cernen t. ncc. which is assuciated with aEcollol rind tobacco use and is more common in blacks.lsional cot ~ g hvomiting. progressing to liquids. HPI Tl-lc patirnt has been drinking w r y hot tea since hc was E I years olcl and smokes or~e pack ofrigarcttcs per clay. untreated gastrocsophageal reflt~x rliscasr). Hemoccult-pusitire srtml. microcytic anemia. which r~suallyir~trcllves the distal third of thc esophagu.e < ? Labs CBC JPBS:hypoct~romic. cllrvature of stomach with kislulous tract running to uarlsverse colon. Imaging Gross Pathology Micro Pathology Biopsy rcveals a well-differentiated adenocarcinorna with signet-ring cells. Pol. rnicroeic anemia.ge fungacing lesioil on grratci. HPI Thc pain is not relieved by antacids or milk. UGI: 1. a n d a gnawing midepigastric pain of several mnnths' d~u-ation. C11ronic atrophic gaytri [is. The patient has lost significant weight over t l ~ e past fcw m o n ~ h s due to diarrhea after e v r r y meal. aderlocarcinomas nlav hc one of two types: intestinal ancl difftwe. f m g a tirlg tnass p t-qjecting into lrunen : sitnated at distill erld of'qrnmach. #'Ion'. PE Pale. emaciated male i n modcrate dist~. infection with H. raised..ess: left supraclavicular lynph node (\r~'r~t:~nw's NCIDF. c l~ernotherapy. pernicious anpmia. v. S ~ ~ r g e rradiotherapy.vpaid.and may spread trar~speritoneallr. frequent vomiting. L R s normal. Labs CRC: hypochromic.) palpablc. to thc rrvaries (KRUKENRERC TL-MOR). EGD: same. and type A blood are all predisposing risk factors for the developmcn t 05 adenocarciraoma.ID/CC tlrl %?year-old whitc male complains of anorexia. post~usgical gastric rcmnai~tr. 5 > <> * v GASTRIC CARCINOMA . Stool positive for occdt hlood. Treatment Discussion Most cnmmonly found on the lesser curvature in the antrum and pyloric areas. I t most cammonlv spreads hematogcnousI!~ tn the liver.ir. lsc)gastric aspirate 11x5 coffee-ground appearance. D rn rn Imaging UGI/EGLI: fixin m a w in fundus c ~ stomach f with 2-cm ulcer o n n < Cross Pathology I'ostoprrativc specinlerl I-evealsa firm.M. I x ~repcatcd t c. S ~ ~ t g i cresection. foul-smelling stools ( LTEI.-1i s 1 1 1 most ~ common Treatment Discussion benign tumor of the stomach. al Gas~ic lcio1nyom. ? GASTRIC LEIOMYOMA .ID/CC A -14-year-old nrale is aclrniltetl to the hospital follo~~ing episodes uf vomiting blood (HI+. t ) HPE Labs CRC: rnicrocytic.V. circ~rrr~srribecl nodrllaimass within the gastric wall cowrccl ky Initcosa.IAI I ~ U I : S I >ancl ) passi~~ black.NA) . ? E L . N.va1~1xtions have heen negative. hypochromic anemia. Micro Pathology Ill~orliug intrrlacetl bzmdles of spincllc-shaped ccrlls: nn ctvidei~ce nr anaplasia. g tarry. THe has experienced recurrent painless hematemesis and melena Ii)r sew t-a1 vears. tli/ed mild tenderness ovrr al)clr>ine~i wi thouc rebouncl o r guarding: tlowel S ~ U I ~ ~ S hearrln$ high-pitched tinkles during pain paroxysms. Ileh!dl-ation .~htloint=n: " ~ t r p l n c l r l ~pattern r" or ~nultiplc dilated loops of s m d bowel and multiple air-fluid levels: colon and rectlim gaqless (air in rolnn or rectam wor~ld i~idic.~te an in~csrit~al ilenq): iln rrcp ail.IDJCC A ~lO-vc. PE Labs Imaging XR. J 3 'fluid aild cluctrc~l!re repl. :tnrl incnrucratccl hernia: 1l1crrlosi cornmoil cnusus of large I~owelobslrur-tinn arc carcinoma.lxrn amylnse levelu ilormal. volvulus. intussuscepiion. Twn years ago. Ilc i~r~clrrwerli an emergency appenrlectr~my Li .pitst IS days. . na50pstric suctir~n/ c l r c r ~ i n ~ ) ~ ~ e br-oad-spectm~m saio~~.unclez dii-lph1~1g111.~~--old male p~esellts wi ~h crampkg abdominal pain anrl vomiting of 3 hourq* rlul-ation. Complications iriclc~dr strangulation ~ I C necrosis I of the hc>~vrl ~v. HPI H r also romplains of a n inability to pass stool or flahls ( O I K T I I J A T I For ~ ) the. CDC:/PRS: Ie~iknqt nri s with I~emoconccn tra tion.y. antibiotics: sr1rgcr.r a r-tlpt~~rerl appcndis. Treatment arc Di~cussion T h e most comrnori cause5 OF small howel ohstl-l~rtinn intestinal adhesions secondary to prior abdominal srrrgery. peritonitis. abdominal distention: gerirr. sepsis. . and s i p o i d diverhditis.lrrmenr. Ser. arlrl h o c k . volvtplus.111 luading lo perT[~ratiotl. rc. A11 The chilrl had previously hcrn wrll. surgical ruduction c)i. thme lavers ar-e seen: entering or inner tube. and parsaqe oT'*redcurrant jelly" stools. .aindiciltcdowing t o perfbratinn o r ganFcnc. I-le vomited twice fi~llowing arlmissian. The condition i s as.~ on stool exam. producing "red currant jcll!~" sronls. 's diver~ic~llum.Meckcl . telescoping of ileum i n t o cecum.ilidolnen: ga? i r l small inte.abrlonli1x11 distcntiorl.ued with adennvirus infcc~ions. otiginale near the ileocccal . Hvrlt-nstatic (barium) or pncrl~nalic(air) reduction using an enema. parasites.ti11ning or middle tube. ~ . Child ca-ying ancl scrcarnir~g.al rectal exan~ination. intermittent abdominal pain. prjlyp5.and mirldle togetlzur crtllcd int~zssuscrptum. m d h i s iniinl~rtizarion scherlule is roi~ipletc-. Imaging Gross PathaZogy Micro Pathology Treatment Discussion Atlas Link 7 INTUSSUSCEPTION . with lead points ( e .~bclorn~ tenclcr n and distended. XR.hich produce liyperplasia of Peyer's patches in t l ~ c terminal ireurn. no pathogrn on ston7 cultrlre. Labs Ko pm-asiitr. Ninety-five pcrcrn t nf case5 o f i 11 tus~usccptionare irliopa~liir and usuall!.jurlctio~i. innri.ing operation. with knees dr. Ischemic necrosis M ~ I F I alcn~ghing of rnucora.lr\n In al~domcn. a n d suture lincs) . and sl~eatlr or cmter. I~cmar~gioinas. Zh~r. refertion ir that fails or is cont~.itirlr ancl a h ~ e n c e t>Ccccalgas s h x r l o ~TSE: . cluplicatinns.18-monthsld male is bro~ig111 to t11e emergency room by his parent? becallsc of acute. ~. which s e ~ ~ as es r7 1 1irll~s for i ~ ~ t ~ ~ s s ~ ~ c 11 ~i~ p also t i u seen r i . .tube: orltur ~ u h e rallerl it~tussuscipicr~s.ioci. oblong (saurage-shaped) mass in atwIornen ( rnr~st often in right uppcr quad[-all[) that hardens wirh palpation: exartlir~ing finger stainctl will1 mucus and blood o n digit. Markedly elevated direct bilirubin (20 mg/dL). chronic pancreatitis. Pancreatic carril-loma carries a poor prognosis (8.lare already locally inv-nsivt. I:ornplica~ions i t~clode I~ypt-1-c~qiitahilitv (resulting in migratory thromhophlebitis.rl nodular mass lui th ill-rlefiiied Ircjr-rlers invading parlcreatic pwenc hmla a n d obsfructing c o m m o n bile duct around Iirad of' pancreas wit11 Inca! extension and livcr metastasus. rnildlv c1t. supportive and palliative care (hiliary decnmprewinn to relieve-jaundice.iriI is.lsiir strnma. (indicate? jaundice). ~urr~bili~lvgen. hepatomeply palpable gallbladder ( C r ~ r r u v c ~ r sSII:Y) i ~ ' ~ ': ~hard 8-cm mass palpahl~ in midepigastric reginn. 'tiureditar? panrr-e. Pancreatic mass biopsy rrcvcals a pclorlv cliffcrcntiated ductal adenncarcinoma it1 cl ilslers.rr. dilated intrahepatic bile ducts.] ~o~.vatt. r.5% . He admit? to a history of smoking (60 pxk-ycars) and Ilea\? alcohol u\r ti111 multiple prior houb of pancreatitis. CT/US: mass in head of pancreas.). Labs Imaging Gross Pathology Mino Pathology Treatment Surgical pancreat icnr[r~ode~zertomv ('M'lit wtr's I'ROCEDL'RE) . or-nle~astatic i i lthe time of rlingnosis) nild is associated with a mutation in the I<-ras oncogrnu ancI thc p53 tumor suppressor genc. celiac plexus block for pain). scleral icteru. diets high in meat and rat. Hitr. also known as thc Troussear~ 5ign). ERCP: abrupt cutoff of main pancreatic duct. xnrl o c c ~ ~ p a t i n nexposure al tn c a r c i n o ~ n arc s prrdisposing factors.ID/CC 1% 3 1 -y=al. cigarette smoking. cIlr=lno~hcrapy. PE Cachectic malc.-old malt=complains of pruritus and abdominal pain that radiates to his back along with significant weight loss (15 kg) over tTic past 4 months. secrw ing r n ~ ~ c ar~rl i n rlen~e collagenmlq r3esmopl.s. HPI H c also status that his urine is dark a n d tIiat his stools are claycolored (A(. ahscnce of ~ ~ r i r l . normal P T elevated caccinoembryonic antigen (CEA) and CA 19-9. PG-P2-010 Discussion Atlas Link ? PANCREATIC CARCINOMA .) markcclly elevated alkaline phospharase.. diahetc? mcllitus. LTGI: ilarrowerl lumen ol'duorlenllm. Chronic galIhlaclder disease.d tri111sarni1iasr. Treatment Snrgery for c u ~ ~ g c t a n lihean tlefects and ctuodet~al atresia: tr-ai n ing i n specialized groups.AGKOMICRI. it is lcss co~nnlonly caused by rnosdcirm or a Rohertsonian 1 randocatinn.POTS).XX. D dngle transverse palmas crease ( s r ~ r .no~idisjancrinn). tlrpal-tment for a karyotypu study. \ u c : ~ ~ ~ ) :swidely i . a highcr incirlt.~tiun for prenatal screening). silverwhitt. her pregnancy was rinrvcntful.flattcnrd r~asal bridge a n d e p i c a d d folds. Discussion The most common chromosomal disorder.. HPI PE Labs hr-yotype: 47. Generali7erl hypotonia. . small brain with shallow sulci: hvpoplasia of frontal sinuscs.ilce of cardiac defects.spols (In rhe periphrry of irises (BUL~SI I ~ E ~ . bisomy 21. Imaging KUR: double huhble (rlilatcd stomach and proximal rlonden u m ) clue to duodenal atresia. 1 I i 5 associatrd wirh a higher incidence with advanced maternal age (i~ldic. plain: hypoplastic middlc arid terminal pl3alang~s or firth digits (. XR. endstarclial cashic~rr derect. pkq DOWN'S SYNDROME . She was Imrn or a 45-year-oldmother w h o f c d s t1lat her child i s developmentally retarded ~vith characteristic "mongoloid" facial features..I DJCC A newhorn fir1 is brought into the genetic. Dorm's syndrome is m o ~ I'requentlv t car~secl IJT trixomy 21 ( r l ~ ~ to c .\). eqpeciallv cndocardial cushion dcfccts: ancl a higher incidencc of acute Iymphocytic lenkemia ancl presenile dementia of Nfieirner's type. Gross Pathology Rrachycephalir tlrad. flattcncd facc and low-set ears: macroglossia. split fixed S2 {dliu to an alria1 septic deCert). Aar feel (1. Imaging Micro Pathology Treatment Spinr bracing. rupttrre. slender Fingers ( m c . endocartlitis prophyli~xis: P-adrenergic blockers.rnyc~pia. Echo: r n i m valve prolapse.~:s IJL%NUS). plain: ihoraciu and lurn bar kyphoscoliosis.. sr\fert. I-Ic i s r+cFerrrdto his family doctor.rr 1 IUSTENCIS~ELL~X) : long.ID/CZ A 7-yc. a r m span greater than hciglrt (oor.aortic va11-e replaretn~nt. uphthalmologic correction. Mnrfan's !iyndi-nrnu i? dne to a defective chromosome 15 fibrillin gene.~ TI^) . crnph~sema formation.t. C y d c medial necrosis of anrta may lead to dissection. high-archrd palate. T h e boy has an ~ ~ n u s r l I~ody al haI~it~is ~ i l long h arms and leg?:a Family hir.~ ) dislocation of lenses ( ~ r r m ~LEN r. IM2-011 fi MARFANfS SYNDROME . C?IR/CT/MR: marked dilatation or a5cenrlin. XR.toryreveals similar body proportio~ls in o r her familv rncrnhcrs. 1 r v o n ~ c m . elastic lung fibers lortllous and thickened.ltrim: scoliosis of ~horacic spine.ni. or aortic insttfficiency. aneurysm.a glvcoprotrin secrcted by fibroblasts that acts as a scaffolding for the Discussion deposition Atlas Link F 1' 1 I I of rlastiri.rsscs.i1rulclbou i s brough to the optnnietrist fnr diminished visual acuity and requests a prescription Tor cycg4. A svsternic conneclive tisnie disease characteri7~d hv an autosomal-dominant pattern aC inheritance. PE 'Sa11: long extremities. inguinal 1ie1.aortic diastolic murmur (anriic insuficiency): funtlel chest due to pectus rxc. Labs E klcreased urinarv h ~ m x y p m l i n e . who on carcfill c l ~ t e e f r i ing o discloses thi11 at1 uncle died nf a ruptured aortic HPI aneurysm.w.gaorta. . Remission Treatment induc~ion and ronsnlidation chemotherapy. PE Labs CRC/PHS: nnrmtrcytic. It c. and bone marrow with l o s s or normal mchitect~lre. She ha5 a history of progrcssir. pallor.A 5-year-olcl white frnlale is h~-ought to !lor pediatrician hecause of h e r . IIOI-ma1 Funduscopic exam. Imaging CYR: no lymphaden opatliy. Discussion the most common pediatric neoplasm.ely irlc1. anemia ~ 5 t h hlood irat~sfi~sionr. PE: marker1 pallor. bone pain. ecch ymrrtic patches over skin .easing farigahiliq :and rearrent infections over the past few ~ ~ l o n t h s . condensed tllromatin. Treat infection with a11 tihinlics. slight hrpa~osplet~umegaly with nontender lymphadenopathy no bigns of meningi lis. absolute lymphocytosis with excess blaqts {> 30%) and neutropenia. VS: fwer. and scan 1 c v t o p l a ~ ~ .tcmlinal dec)?. thrombocytopenia wit I1 pla~elet roncentrations.y~ransferase (TDT) positive (marker of irnrnatlire T' anrl B lymphocvtes) on c n m c marker st~trlics. sternal tenderness. llormochrornic anemia. negai ~ V PInnnmpnt test fix Epstein-R~rr virus.irries a good prognosis. epistnxis. . Iynmphoblast~ with inconspicuouq nurlenli. and hleetling from her HPI nn5e ( T : P I S T . thrombocytopenia. ~ I S ) . L E U K E M I A (ALL. Coiidder hone marrow transplant. Common acute Iymphoblastic lcukemia antigen (CALLA) (CD 10) positive..i t accounw for 80% of all childhood Icnkcmias. Gross Pathology Micro Pathology Myelaphthisic bone rnwrow (distorted archirect~tresecondary to spare-occupying Irsions) with l~mphobla~tir infilmation.. marked weakness. spleen. Acute Iyrnphocytic loukcrr~ia(ALL) i s Atlas Links Lq ACUTE LYMPHOC. liver. Neoplastic infilrr-atinnof lp~nph nodus. and marked weakr~esq. menorrhagia. hepatosplenomegalv. H-P2-014B. basophilic cytoplasmic bodies (ALTER RODS) in mvelorytes. early blast cells): neutropenia. sturnal renrlerness. A 1 increased risk is aswciatcd wit11 ioni7ing radiatinn. Acu tc mvelogeno~~s leukemia (AWL) ic no1 as common in children as i s ALL. ~nainlv in skull. she has also hacl recurrent infections.ic rlirrerentiation replace normal mar row ( m r . Discussion Atlas Links !TZEZ H-F2-014A. henzcnc exposure. CBC/ I'M: normocytic. nt3rmal fundr~scopic and t~clurologiccsarn. Gross %thology Bone ei. ~ ~ u l t i p I purpuric e patches over skin. Micro Pathology Treatment Chcmothe~apy: dl-trans retinoic acid in acute promylocytic Irukemia. bTy~lohlastr with myc~urnonocyl. Over he paqt seve~xl wccks. spIenurnegaly. HPI PE Marked pallor. R-P2-014C. leukoc~~osis composcd riiairlly of myeloblasts and prornyelocytes (rrnnmnn~ring. thrombocytopenia. peroxidase-positivestains o r 1 bone marrow a n d gingival biopv. H-P2-014D ACUTE MYELOGENOVS LEUKEMIA (AML) . Prolongecl F T ' and Labs m. gingival hyperpIasia.nsion clue to marrow expansion.3 r d cytotnxic cliemotl~urap~lilic agents. ~ o ~ t i r HONF ~l~r M c AKKOW) .IO/CC A 25year+ld woman prcsunts with high-grade fever.chloroma formation. bone marrow transplant during firfr remission if HM-maiched donor availahlr. Tlow~l's ~yllclromu. nomochl-omic anemia. g.~). fibrous stroma. no hepatosplenamegaly. Atlas Link r " l H-P2-015 * + ANEMIA-APLASTIC . he has a history of recurrent UlUs ancl high-grade fever that have heen rreatrd with parentcral arlribiotics.. and thrombocytopenia ( P A K C ' ~ T ~ ~ P F .. markcd pallor.juncriva:nral and nasal mucoaal petechiae. with low rrticulocyte cotlnt.GM-CSF) fhr- Treatment neulropenia. nnr~nal RRC morphoIogy. negative Conmbs' test: normal chromosonial sturlies. purpuric patches visible on skin: no significant Ivmphadenopathy. Gross Pathotogy Increased ycIlow rnarrow and decreased rr-ct marrow. Other causrs include viral infection anrl Fanconi's anemia. hnne rnarroiv transplan ~rtion: immunosupprt~ssive acatmen t with antithyrnoqte globillin : niyeloid growth Fdc tors (e.. Mirro Pathology Hypncellular hone marrow with empty spaces populated by fat cells. hcnzcnt.ID/CC A 12-year-old male presents with high fever.chloramphenicol) . neutropenia. benzene). -4plastic ancmia following $rug or toxin exposure may be close dependent (e-g. Discussion Sixtv-five pel-cent of cascs are idiopathic. and scattered lymphocv~es. radiation) or idiosyncratic (e. an autosomal-rrceqsivedisorder in DNA repair. markcd decrease in all cell lines. PE: marked pallor of skin and con.. and epistaxis. cytotoxic drugs. He has also sho~yn marked weakness over the past 3 months. Normal seri trn bilirubin.g. N anemia ~. He lives in thc vicinitv o f an industrial itnil that handles petroleum rIisti!lates such as benzene. HPI PE Labs CRC/PBS: anemia. VS: fever. Retnoval of myclotoxin (in this case. immuno~upl>r-es~ive Efrt~gs. Three main types ex is^: warm antibody (80% tto I)O%: assnciarerl with lelikemia. positive Coomhs' test. seticulocytosis. PE: dyspnea. and \-iyjr. and dn~g-ind~aced (methyldopa. transfi~sions: sple~~ectoms. PE Labs CBC/PBS: severe anemia. lytnphorna. Discontinue any offending drug. pallor ef skin and mucous membi. compIemen~ activation.ssivelv turning dark and red ovcr the course of the day.ll infrctions). "'hite cells. slight jaundice. Congestive splmomegidly (due to extravascular hemolysis in Ihc spleen). sphemcv~osis. splenomegdy. it is characteri7erl Ily autoanti bodjcs against RBC mcn~branes (Rh} .-4 ti6-year-olcl white m a n recently diagnosed with chronic lymphocytic leukemia cornes inlo he errlergency room complaining or fatigue and ~achyca~dia. Prednisone. cold reacting antibody (1 0%. HP I He also states that his urine I-tas been progrt. qllinidirle.ar1~4. Gross Pathology Treatment Discussion Atlas Link 9 ANEMIA-AUTOIMMUNE HEMOLYTIC ." LA: lternnglobinuria. associated with EBV/rnycoplasma infections and lymphoma) . and phagoqtosis of RBCs bv splenic macraphages. SLE. Tnci-eased serum indi I-ecrbilirubin. A4utoin~mune hemolvcic anemia is idiopathic in a b o ~ 1 ~ 5 0 of % cases. penicillin). VS: mchycarclia. reddish-blue rnottling o T skin in fishnet pattern ( z . the foun-th reculted in unexpected fetal death. cerebral artery ocdusion associatccl wit11 strokc and tranqient ischemic attacks (TI&). m n o RI:~CL~L-\RIS)on cxtrc~ni ties. it is li~rther recurrent deep venous thrombosis in thu lower cxlreniities. either alone o r in cornhination wit11 prednisone. i11 right Treatment Anticoagulant therapy 74th heparin: uqe of lowdose aqpirin and heparin.c~rnbosiq in t l I-endl ~ and hcpatic veins. or cliabrtus and nuiehcr smrkcs nor lakes drugs. n o carotid h r ~ ~nn i~ cardiac : tn~innnrs. SpnntanPnlIC ahortioris 01. ~hr.intrauterine clurnisc). The prewnce nf lupus anticoagulant and ACA defines characterized by antiphospholipid syndrome. PE Labs CRC: rnilcl thrombocrytopenia. Imaging CT. Her first three pregnancies wcrc spontaneously aborted. Prolonged nor-ma1 bleeding and clotting times.l'ssrm) : no nrck rigidity fundtls normal. left herniplegia with exaggera~erl d e ~ tetl~lor~ p rellexes i u ~ d extensor plantar rcspunsc ( P O S I T ~ TBmm.< I:18): FTA-ABS for syphilis negative: ELTSA ~horus presence of anticardiolipin antibody (ACA). false-positive VDRL (titel. HPI Shc has no history of prior hcadachus. hvpcrterision.. PE-: patienr r o n ~ c i o r l mikt ~ : pallor. VS: normal. scizurrs. positive I Tomans' sign in left leg. t~exrl( 2 4 hours later): hypodcrlrii~ ( d i ~ to e infarrt) internal capsuIc. and neurologic tinding5 that resrmblr inulti-infarct dementia or epilepy- Discussion * ANTIPHOSPHOLIPID ANTIBODY SYNDROME .g. pulmonary hypertension.sw.ID/CC A 35-year-nld w m a n is admitted to the hospital with left4ded weakness upon awakening. is atlvocated during pregnancy in case5 ~i th a complicated obstetric history (e. b u t no ulceration or uccl-osis. inrrathecaI metholrexare Tor meningeal prophy1. high mitotic index and typical "starry sky" imagc pattern ( d u e to cliff~~re disrrib~~lion of macrophages arnrmg tumor cells). endemic (hfiican) fonn is cha~~ctcrjze b~rjaxv d tumors and is assoriared wilh EBV infection. presen w with a largc swelling of the left side of her face and jaw of 3 weeks' duration. Rillor. Discussion Atlas Link * A - BURKITT'S LYMPHOMA . thc daughtcr of African immigrants. posi~iw direct Cnombs' t a t . she c o m p h i n e d of loosening of the upper ~ccr~n left d molar. prodttcing mild ipsilateral exophthalmos wit11 deformation Labs (311 left side of lace. and evenly distributed chromatin surrounded by small. t h c noncndernic (M1estern) Term i5 characterized I>v atldominal anrl pelvic involvement. Giemsa-~tained FNA show cells of uniform size with iloilgtanulalhasnpl~ilic nucIei and some vacuoles. there is no pain a~soriated with it. mild leukopenia. ~ h i n rccentric .k ii ~ n t1958 in Uganda. cstoplasm that is pyroninnphilic. Micro Pathology Treatment 14igt1ilose.14) ion involving c-myc gene. normochromic anemia. Karyotype: chromasomal ~ l ? u ~ s l o c a ~ t(8.A 9-year-old girl. CBC/PBS: nurmocytic. Imaging ( X U :nn evidence of mediastinal widrminl: (vs. Burkitt's lymphoma is a small n o n c l ~ a ~ e lymphonla d (non-Hodgkin's lymphoma). Hr>dgkin's Ivmphorna). Gross Pathology Firm. ill-clrfinetl maw el~cnmpawing e n tire upper mandible. Ilnrle inarrow rransplantation.~~is. large. 2 to 5 nucleoli. It is a pclorly dirferentiatcd &ell IynphohIastic l y n ~ p h o m aThe . ill-cletined rumor involving upper mandible ancl defotmi ng neighhori~ig 5trl1chires. short-term chemetherapy: rtlkalini7e ~~rirle. firm. Thc condition was first drscribed by Denis & u ~ . no satellite adenopathy. Trvn weeks i l p . Duspitc the sizc uf the tumor. Cnl-ce rliuresi%. ?~v with tumor r-lndule fol-mariotl. mild rhrornh(~cytopenia. enlargement of spleen and liver.ite lymphocyt~s). Iyrrlph node enlargeinen t. togrther with upisucles of epistaxis and extreme Fatiguc. and bleeding episodrs. Chronic lymphocytic le~~keinia (CLL) is a malignant neoplastir disease of R lymphocytes that exprers r h e s~lrfkce marker CD5 (usually in T lymphocytes). s p l r e ~lymph ~. smudge cells (fra~. I~cpa~osplcnornepl. Imaging Gross Pathology Lymph node enlat-~ement i~lrno~t always pr-csmt. PE Generalized ~ ~ o n t c n dIymphadenopathy: cr pallnr. Labs CBCJPBS markedy elevated WBC cormt ( 1 24. Bone marrow biopqy reveals es~ensivc. liver.000). Coomb+positive hemolytic anemia. recurrent infections. round nuclei and scant rytoplaqnl. mainly by normnl-lnoking lymphr)cytes and a frw Fymphohlastq M-i th small. dark. infiltration. I~e~noIytic anrmia. Discussion Atlas Link C H R O N I C LYMPHOCYTIC L E U K E M I A (CLL) .-ltions such as autniininur~e l~crnoly~ic ancmia or immune thrombocytopenia. 90% lymphocytes. nnde involvetnenr common: I3 Iymphocvtcs fail to mature Micro Pathology ~'"Pc'IIc Treatment Cf~errlotherapy.A 65year-old male visits his ranlily doctor Cnr a rol~tine a11nual check~tp. no lymphohlasts. he admits to a weight loss ofahout 12 pounds over the past 4 mnn I hs. it iq cl~aracterizcd by slow progression of anc-mia. prcclnisone or spler~ecrotr~v for complic. On rlirecterl hisiory. Micro Pathology Hep. hrpatornegaly ( d u e t r> pro1iFeralion and infiltrai ion bv grannlove precursors and maturc granulocytes). US. elcva ted serum vi t anii TI B .ceIet. .I. The patient has no majnr complaint5 except for occasional fatigue (due 10 hyperm~taholic stale) and increasing abdominal girth (clue to enlarged spleen). rw-interferon. In chronic rnveloge~locis leukemia (CM?. leukapherr5is.l~ed tiansformation into acute leukemia (RUW cnrsrs) within 2 co 5 yean. Imaging Gross Pathology Skull chlorotnas ( ~ n a l i g n a t. Lxvoqyt: chromosornnl tratlslocatinn t(9:22)/bcr-abl gene (PH~L\I~F. ~greencolored l tnnlor arising from ~nvelnid t iqsue) .A 40-vcar-old white male visits a doctor fur a life ixhsti!~ance pllvsical examina iioti.~~kc 4tlusoirIaZ Iruk~mic inlilrrateq: congestive splenomegalv 1vitl1 rnyeloicl metapbasia. bone marrow tran5plantatiun {the crnly potentiafIy curative trealtnent ) Tteanwn~ ineffective afrpr development of hlast crisis.000): itnmatore gr. early ~ I i r o m h o ~ ~ o late ~is t: hrnml~oc~~opcnia.). zlhdorne~i: splet~oinegaly. Labs CRC/FBS: markedly elevated WBC count ( I 30. eosinnphilia. PFliladelphia chromosome in all myelnid pl-ngeny. Icvcl. Treatment Discussion Atlas Links 7 C H R O N I C MYELOCENOUS LEUKEMIA (CML) .PFI~ 4 I:HKOVOSI)MI:). clearh us~~allv res~~lts frnrn ac. nr) Ivmpha~Eenopathy: 1 1 0 othcr abnormalities found. pain on palpation over sternum (due to tna rt-ow nverexpansion).inuioq~cs mixer1 u i ~ h normal-appearing ones: basophilia.it~es: markedly enlarged spleen. Low lenkoqte alkaline phosphatase. enlarged and congested spleen with arcas of throinhosi~ and microinfarcts. Hydroxvurea. Pallor of skin and inucolis mernl~r. pain eIicited on calf palpation and on dorsiflerbon of right foot (HO~L S' Y SICY) . 4 " C : ) . Appl-nximately 200.i LII TV heparin. Doppler: thrombi occluding right cornmon Femoral and popliteal veins. but rarely indicated. Imaging Treatment Anticoagulation u. C:omplications of DVT inclttdc ptllrnonarv embolism and venous ulceration. notrnal Rn. and redness of the right leg. PE Labs Blood D-dimer elevated.ID/CC A 55-year-old male presents with swelling. HPI: He is retired and leads a sedentary lifestyTe. Cnlln~ved l ~ long-term y an~icoagulation wit11 oral warfarin or subcutancuiis lowmol~cutar-weight heparin. and hypercoagulabIe state) contributes to the furmation of vrnous thrombi. PE: right lower extremity swollen. S~ircliow>triad (venouq staslis. mild hyperterhsic~n (BP 142/92). tachycardia (HR 106).000 deaths per year in [he Unitecl Statcs arc attrill~itahle to pul- Discussion *< DEEP VENOUS THROMBOSIS . 1 ' s : fever ( 3 8 . Hc admits to a 70-pack-yeas s m o k i ~ ~ history g and occasioi~al alcoIiol in take. Venography: <goldstandard for diaqnosis. pain. vessel wall injury. US. and insufficiency. . fresh fyo~en plasma: fihritlogen cl-~opretipitare: platel~rs. Mx~iual exploration ofthe uterus iox~ealssetairred placental iis. Treat underl$ng disorder. parn-negative septicemia. limb ~hrornhosis with pang-cne. r)issernina~ed ii~travasc~ila~coagulation (DIC) is a Illeeding disorrler that is clue to cons~~mptinn of plateletc. burns. bleeding diathesis of skin (huth petcchiae and purpura) with oozing from venipuncture sites. and cr>aplatio~l. b y cancer.lie that requires rlilatation anrl curettage. fibrin. elevated fibrin split prodtlcts. Treatment Discussion DISSEMINATED INTRAVASCULAR COAGULATION (DIC) . 50 minz~tcs after thr procedure. and abstetric complications. and ischemic adrrnal necrosis. mu ltiplr trauma. Cross Pathology May see ctxnplicaltions such as renal cortical necroqis.. l e a d i t l ~ to microinfarcts in practically anv organ : also hemorrhages and pctcchiae in ini~olverl organs. csprcially Ddimcrs. CIIC: low platelet count. the patient hegin5 tn bleed prafwely from her gums and conritlues to b l e d vaginally. factors secondary to excessive clotting in niit:rocir~c~~ia I t~is i (precipitated ~~~. Diffuse hlceding in gums and oral rnucosa. Prolonged PT and activated PTT. Labs Low fibrinogen.I 25vea1--oId white fernale continues to bleed sreadilv after a nartnal. spontaneous vaginal delivery. arninocaprnir acid with heparin. Micro Pathology Microthrombi in astl-tioles and capillaries. and HMG-CoA redudaqe it1 hi hi tom (statin drugs) may he nsed. in resis~nn 1 cases. HPI Ilcl~asafan~iEyhistoryofpremah~reatheroscleroticcoronary artery disease (CAD). chylomicrori remrlaills present i11 f'ds~i11g plasma. coronary: arherr~sclcrotic coronary artery diwa. clec~t-ophor-esis reveals beta migrating VLDL:isoelecrric t'ocuqing shows ETI/ETI genotype (nearly pa~hognoitini~ic) . PE 15: mild hypertension. CIiaractcristic atherosclerotic plaques.~nclknees) . .I<UII'I. tuhoeruptive xanthomas on pressure sites (elbows. avoidance of nlcohol and other trig1yccricle-raising drugs. high-dose nicotinic acid (niacin). regular exercise.I?N. Weight reduction t o ideal body weight. wuak peripheral pulses. Rela-WADI. buttocks. palrnar xanthomas and tendon xar~ tl~omas: orange-yellow discoloration of palmar (pathognomonic for dysbetatipoproteinemia). triglycerides.particle5 are chylomicrons ancl VLDL remilants caused in part by a mutant apo E rhal impairs the Iiepatic llptake of apoproteitl-Ecnntaining lipoprnleins (Vl. low-fat. Imaging Gross Pathology < . a n d c hy/lotnicroilf). ?Ingio. Yellowish in tralutnin a1 atherosclerotic plaques sccn in tlze aorm and rx her h r g e V P S S ~ ~ F .ID/CC : I 35-year-old man corn plains of pain in his calf rn~lscles while walking that is rclieved by rest (INI+. creases Labs LFTs normal ..f)l.I C I A I~UICATILIN) tc~gett~rr with exertional rIlest pain. and VLDL and reduced LDL and I-FX)L.e confi~-med. gernfibrozil. lipid profile reveal5 elevated total cholesterol. PE: obese. 0 z r) 0 GI Micro Pathollogy Treatment 0 r o G) < Discussion @sbetalipoprotein~'mia (TYPE rIr I ~ E R L I P O P R O T E ~ E ~ M is) clef ned as the prcsunce of VLDL particles that migrate to the hela position on electrophoresis (normal \%DL particles typiral ty migrate to rhe pre-hpta location}. 7 DXSBETALIPOPROTEINEMIA . Irw-cl~oleskroll diet. CRI: JPBS:anemia. Dcoxvcoforrnycin and =-inturforon are highlv effective. Discussion Hairy cell lc-ukcmia is a chronic B-cell malignancv.41RY CELLS"). PE Labs Imaging CXR:normal. Pallor. gingival bleeding. inclirrling vasc~lliiis and arthritis. thin cytoplasmic prqjections ("I 3. I t i f alqo characrerized hv atypical rnycobacterial infections. DTTZ H-P2-024 Atlas Link 7.\). lymphocytes with characteristic long. or iiscites. marked splenomegdy: mild hcplziurnegaly: no I p phadenopathy. and hone tnarrnw infiltt-aterl Ily leukemic cells. and an abdominal mass. large proportinn are hairv cells and contain tartrate-resistant acid phosphatasc (TRAP): splenic biopsy rrvtxls Ieukerrlic in fill ration of red pulp by hairy cells. Gross Pathology Liver. icterus.~NCYTOPENI. decreased WBCs and platelets (~. atitoitnmune svnclrr)mes are frequently seen. ? H A I R Y CELL LEUKEMIA . CT/US. &one marrow largely repIaced by leukemic cells ( M +TIOPMTI-IISIC ROVE MARROW). Micro Pathology Treatment splenectomy. n o Iymphadunopathv. ~pler~onlegaly nrav he significarlt.ID/CC A 61-vrar-old white male presents with marker1 weakness. mild hepatomegalv. HPI He has a history of'recurrent bacterial infections and has no1 u-aveleil nuuide thp United States. no evidence of portal hvpcrtension. spleen. ahdompn: massive splenomegaly. PED-019B 7 HENOCH-SCHONLEIN PURPURA . normal coaplatioll tests. He has a history of allergy to dilsl and pollen.cr buttocks and legs. abdominal pain. low-grde fever. RECs and RBC c a s t s . Cross Pathology Micro Pathology x rn OkAA 2 3 r o 0 G) Treatment St~ppotlive.on urinarv serliment. Nec~mtizing vascuIitis of k i d n e y and lungs. s~eroicls. mesangfd IgA deposits on immunofluoresclmcc. painful restriction of knee and ankle join1 movement with swelling. Renal biopsy s l ~ o ~ Ibcal vs and seg~netlta! glomerulonephritis ~ vth i crescents (11lesangioproliCerative) . G I Atlas Links FEPP PED-019A. Increased ESR increased BUN and serum creatininc.ID/CC An 8-yearald white male presents with an rr-ytheinatous skin rash over the buttocks and legs coupled with joint pains. ancl hematuria.~. Labs CBC: normal platelet count. coryta.i s a common vasculitis (s~nall vessel) in children. HPI Threc days before h e had complained of cough. and sore ihroat. irliopathic disotcler that is also known as anaphylactoid or vascuIar ~ j ~ t r p u rit a . UA. PE: palpable pwpuric skin lesions o. high-dose irn1n\rnnglcrhu9i 11 I herap7 U Discussion HennchSchrinleitl pul-prira ir: a generally selr-lin~ired. PE VS: hyprrtension. Positive stool guaiac test (due to orcaI t blnnrl). . and spine are cornrnon locariotls. The skull. h ~ t twithout ailv systemic rnanfisla~ions. CkT: punched+ut lesion in foul-111rib otl Tight sicle. The child is otherrvise well and is growing normallv. pathnlogic fixture. histioqtosis. it is an inclolcnt disorcler that affccts cliilclren and young adults. 1Gyear-old male i s brought to a specialist bv his parents clue to persis~rnr pain and tenderness on the right side of his chest of a few months' duration. Brownish granulation tissue containing abundant foamy histiocytes ant1 eosinophils with leukoryte5 and giant cells. Labs Imaging Gmss Pathology Intrarncdullary expanding. espe~iiillvmales. I n some cases tt~el-e may he spot-lmnenushealing nl. Discussion F. surgical curetvage mav accelerate Micro Pathology Treatment healing. Solitary Imne leqicltl.ocinnphilic granuloma i q x rvpr of l a n g ~ r h a n ' cell . Ro1rrin. mandible. eroding lesion.E O S I N O P H I L I C GRANULOMA .lihrosir. The disease mav xlqo he multifocal. within a period of 1 to 2 veal-s. HPI Therr i q no history o f tmurna to the affected area. Diagnosis i s IxtserI on radiopaphic dernonsfrariot-i ol'a localized destr~~ctive lesinil arising Crom inside the marrow cavity. involving the * HISTIOCYTOSIS X .c la11 przrameters normal. in w m e instances. PE Exquisitely teilrlur si~ef o l ~ n d overlying ff~utth rib on right irk anterir~rly: remainder of exam ~mrumarkable. Lcsions rcsolvc span taneouslv.ID/CC .: may be asymptomatic or rnnv c a m p pain and tenderness and. infections. positive $1 OO protrin and CD 1 antigen. mild hepatosplenomcgaly. Treatment Cornhiua~iotl chemotherapy. Labs CBC: normal blood counts. skl~ll: multiple rounded lytic lesions. Low weight for age. I-Tancl-5chiiller-C:hristian syndrome is nlultifocal.VOS) and excewive urine volume I~OL~YURIA).ID/CC A 2-yea~rild boy is hrought in for a pediatric cons~~ltatirin because h i 5 parents are concerned ahout the child's protruding eyes (EXCJPAI'HA I . bilateral exnphthnlmns: painful swellings over head (due to cystic bony Iesions) . ci ire1 lase of bony le\ic>nq. Discussion . producing diabetes insipid~~s d u e to thc involvement of [ h e hyporhala~nus and exophtl~. decreased urine osmolality. no PE lymphadenopathy. Imaging Micro Pathology XR. no icterus. Bone Iliopsy Ft-orn skull lesions sllow gr~nulnrnatour lesions and characteristic Langerhans cells with coffc-c-bcan-shapednucIcj and pale. HPI The parents also state that the child has hcrn febrile and has harl multiple rat. ahundant cytoplasrti: tennis-racket-shaped tubular structures ( ~ R R I < C a K w r ILKS) nn elec trnn micl-o~copy. A type of Langerhan's cell histiocytosis. lncreaqed serum osmolality.+lmc~s trorn orbital infiltration by histiocyies. It shows sinlilari tics to acutc leukemia and r~thrr infectious processes.lchl. chemotherapy.OPKNI. It is marked by revet. X R cystic. surgery or radiotI~erapy for lnralirerl h ( n n disease.h 2-yearald wl~ile male child is seen with complaints of fever fc~llrrwed kv a &ffuse skin m h . t hcse cr llr: were further fbund to I>eH LA-DK-pa9itir. Corticosteroids.c. n i a h e t ~ s insipidlts. Skin shows prese~lcc~Textensive eczematoid rash. Labs CRC: ane~nia: thrombocytopenia ~ vth i Ielrkopen ia (PIN(:YI. ) generalized Ivmphaclenopathy: hepa tosplenomegalv.lIrnos.e and expreqsing CDI antigen. large destrtlctivr hone Icsiuns found on sku11 and pelvis. otc>scopyof left ear reveals cl~ill. Gross Pathology Micro Pathology Eosinophilic grantdomatous lesions in aIl involved organs. abdomen: he pato splen omeraly. 1"1 HISTIOCYTOSIS X-LETTERER-SIW E DISEASE . exophth.fever.EM 5h o w cal Langerhans cells with characteristic Birbeck granules.~r+ulopap~~lar ec/ernatous purplric skin rash and s~~hsequent l~epatosplenomegal anrl generalized lymphadenopa~hy. Imrn aftrr an uncumplicated pregnancy and delivery 17% t. rarefied lesions on skliZI and pelvis. poarlv n ~ o l > itympanic l~ membrane with pus behind i t ([I rrrrs ~ I I : I ) ~ A. and hone lesions are ii~uallv wen in conrhinatiot~. diffuse maculopapular c8czematoussash. The chiEd wxs apparently/ we tl a month ago. lmaging CT.drw to Incalized infection followed by a rliff~lse m. PE: mild pallor.Z):t-rlative eosinophilia.~rctia. ~ Treatment Discussion Lrttcrer-Siwc disrasc is an acutc o r subacute clinical syndrome of unknown ctiolop affucting children less that1 3 years old. ID/CC ,A 24year-old white male complaiiis of rapid enlargement of his abdomen, producing a dragging crnmuon, along wit11 a painless lrmp in his neck fnr lhe p ~ q 2 t months. HPI The palien1 also coinplains of iiltcrmittei~ t fever, drrnching night sweats. pruritus. ancl significant weight loss. Paltor: unilateral nonterrder, rubbery, enlarged cervical lymph nodes; splenomegaly; no enlargement of tonsils. PE Labs CRC/PRS: neritrop hilic leukocytosix ~ i t 1'1t1npliopenia; h normucyric anemia. Elevatccl ESR: elevn~ed serllm coppcr and ferri tin ; ncgativu Man toux e\t. Imaging GTOSS Pathology CXR: bilateral hilar lymphadenopathy Itlvolvud Ivlnph nodes arc ruhherv and have "crlt-potato9 appearance of cut surf'acr. 1 rn Micro Pathology I.!mph norlc biopsy shorrs large h i s ~ i o c y t ceIls ~ with multiInhed nr~cIci ancl eosinophilic 1111rleotus resembling owl's eyes (REF.~-S~T.RNRFH~; I .t. i 1: 5 ) ; nn honc marrow in~olvemen t on bone marron biopsv. 3 5 \ r 0 m Treatment Discussion Radiotherapy and cl~etnotherapy. Four patterns of Ilodgkin's discasc are seen on lymph nodc biopsy: lyrnphocytic predotnir~ar~ce 5% to 10%: nodular sclerosis 65% to 75% (seen 11-eqnentlv in voung {wmexl): mixed cellularity 20% to 305%; and Iympl~ocvte dcpleterl 10%.Prognosis worsens in this ordcr. Ann Arbor staging 1 1 1 ' with ~uhclassificatiunA {no cclrlsti tl~tional synlp tom%)a ~ i r13 l (weight loss, fcver, nigh1 sweatu) !nos1 acrul-ately predicts prognosis. The disease spreads to contigtrouq lymph nodes before heinatogcnous diaserrlir~a~iot~. O 1!TT R-P2-029 < a z r) o b I 3 < Atlas Links DI7E JMI-049 .a'< HHOGKKIN'S LYMPHOMA ID/CC X 3-year-old white f ~ m a l c i~ hrougllt lo the ~rnel-grncy room with :I skin rash and severe epistaxis. HPI T h c patien1 Zlad a LRI c o i l r i ~ t i n g of a srvverc c o u ~ h and a riiilny norc 1 0 davs before the onset of her symptoms. S h e has no prir~t 11 irtory ot'prolonged bleeding follnrving mininlal trzltlma. PE Mucosal petechiae; episaaxiq; hemorrhagic budlae in buccal ~ I I U C I I S ~~ : plc~' n1 o1 nl~alpahlc. Labs CRC: mild anemia: low platelet count ( I I1.000); RBCs and W C s normal. P~.olnngt=cl I>leedingrime; n o r n ~ a PTT; l nt)rmitl PT. Purpura (tluc- In r ~ t r a v a ~ ~ l iocbt r lllor>d ~ froin inu-ava~cuIar space in 1r1qkin) : piii-~i7erlhemol-1-h:lges (I~I?:;:~J.:CI I 1 , ~ ) : eccl~vmosis ( l a l - g ~rrhxn pui-p~lrx). N crrrn~~ hoilr l marrow aspir;t!e with increased number of m~gakaryocytes. Prcclnisnnc; splrucctc-rmy;WIG I t l i r )1>i11hi< I 11 r.ot~~l)orvtr,pPniT ~ ~ ~ I - P I (II'P) I ~ X is a n a u t o b ~ ~ n e rliw;~sr. 1 1 1 h>r.?n;ltiunof TgG antiplatelet antibodies anrl sul>5equcn1 pIatrIt.1 deqtr uclion in 11iespleen. 1r often folfows a viral inrection all<!i s srlf~limiterl in r.hilclren h ~ r chronic t i r l adults. Gross Pathology Micro Pathology Treatment Discu5sion IO/CC A 64-year-old blztck m;dr nnri casy Fatigability. 1;11ffttr-r frr)iii hone pain,weig11 t luss, HPI PE I I c aIsc>co~nplains 11f reamrent WRrs and frequent nosebleeds. Pallur: bone tenderness in Ir~wer back and rihr: petechiae on huccal mucora: n o hepatospl~nomegalv. Labs CRC JPBS: narrnocytic, normochrnic anemia: ~ ~ e u t r o p ia; en rouleau formation (KBCs adhering together like >tackof poker cliipq). Elevated serum calci~un; normal a1kaline pl~osphatase: mal.kedly increased ESR; gamma spike on serum protein electrophoresis (monoclonal gammopathy) . LTk RenceJones pmteinuria (clue to IgG light chains). XR. plain: punched-out, Epic bone lesions in vertchrae, long bones, and skull (xuial skrlcton). Imaging GTOSS Pathology Multifocal replaccrnu~it of norrr~al bone tiwue rvii h tumor cells (~dasmacyiorna): prlvi~, skull, and spine most affuctrcl. Infiltration of bonc n~arrcnt~ l~v 11osrnal-lookingplasma cells (al~undan t cytopIasm. eccr-ntric nuclei) in ~tggregates; amyloid deposits in kidney with re1131 ~ ~ ~ l - n cast ~la hrtnation r and intcrstitial fibrosis (can causc renal insufficiency);b o i ~ r erosion and dc.?itruction of curtical hime. Micro Pathology Treatment Cliemoth~r-apeutir I-egimen; hvrlu~rion; treat hypcrcalccmia and Ilvperuriccrnia. Considcr palliative rdrli;-ltioirtherapy. MultipIr mvrluma is a primary mdignancy of plasma cells ~i~ljth I-rplacemenz of nurmal bvne tnarrow; i t is [lie most cninmon primal-v brine carlrei: The l~rognosis worsens with anemia, renal failurc. and mr~ltiple lptic lrsior~s. Discussion Atlas Links H-P2-0314H-P2-03 1B "*1 I ' T MULTIPLE MYELOMA Mime Pathology Wry tap" on bone marrow biopsy: l-i>pcellularhone Inarrow (h~-perc~llular early in diseasc) .. giant abnot. low hematocrit. mudera~e amount of aqcitic fluid. shortness of t>reatl~. with sig~lifica~i t increase in size and weight together will1 firm consistel~cy. replaccmcn t of mar row cissur will1 fibrosis (positive re~iculin on silver stair^): preservation of normal architt'c(111.~S teardrop-shaped ~I~IC: FSiCs. Imaging Gross Pathology XK. ~ Treatment Discussion Also callud .ignogeiiic ~nveloid rnetitplasia. immaturr. Massive splenomeply: en larger1 liver. and ligh~l~eaderlness over l h e coursc of aftnost onc ycar. plain: dcrise hones (generalized os~eosclerosis) Extramedllllsuy hematopoiesis.and r~nrmohlitsts src11siinril tat~eously (~. Eie has also noticed a feeling of heavineqs in his ahdornen and increasing girth a? well as recurrenl deep p i n in the legs and occavic~r~allv in the uppcr abdomen. multiple petechjae on thorzcu and extremities. anemia. significant increase in number of ~negakar-yocytes. tnvelofibrosis wilh rnyeloirl metaplasia is an idioparhic canclitiori in which increasrcl S C L ~ L ion ' ~ o f platelet-clerivecl growh factor (PDGF) and TGF-13 catrw% replacement of hone marrow tissue with fibrosis.ma1 platclcts. headache.4 54-year-old white male complairw of easy fatigability.lrBC1. with increasing scvcriy. no lymphadenopathy (one cliffrrential f r a tl~r-e ~l~are with d cllronic mvelog~r~rnis leukemia). whiclr is prorninen t in liver and ~pEern.EL~I~~F.2) . Atlas Links 7 MYELOFIBROSIS W I T H M Y E L O I D METAPLASIA .e I I spleen. Labs CBCJPBS: ancmia (Hb '7. 1. I~I.~Z~THR~ SMEAR). t l i i .Plilaternllv i n his IOJCC A 5Jyear-old whitc male notice5 painless I~tmps neck h a t haw slowlv enlarger1 nvcr the past 3 nionrli%. s the 111r1stcoltlrnon form and are associated wi 1111 (14.pogammaglohulinrmia. 2 r) o r w I 3 < r"C1 NON-HODGKIN'S LYMPHOMA . CT/lJS: I\mphacienopathy: splcr~umc. PE Labs Bilatuml cervicill firm lymphadenopathy.galv. I rn Imaging Gross Pathology Micro Pathology 3 3 b 2 Treatment G) \ 0 i Discussion Primary maIignan t nroplasms of l~rnphocy~er: arise in IyrnpEloirl tiss11eanywhere in the hodv. night sweats.oombsrposiuve hemolytic anemia. episodes of mild fever.lr<lxiceof cut sur-f'ac~. Elevated senm LDH (a uscfril ptopu.h he clunics any pain.. CBC: C. The prognosis is more dcpundcnt un g a d c tIlar~o n stage. HRI AItho11g. pallor. thev occur mainly in lymph r~cldrs h m may involve inu-a-ahclominrrl organs and bone marrow. AlkyTaril~g agents in varior~scombination^. he ad~niw to havit~g. and some weight loss over this period. Lymph nocles havc grayish hue o n outside and "cut-potato" appe. o ! n b n c ~ ~ n p ~ ~ ~ i a . LvmpIl ilode biopw dcmunstrates notl~xlar. r-xrliotl~~rapy if localizrd: bone marrow ~ransplantarion. histiocytiu and strrn cell lvm~>homn. 18) of 13cF-2 (an anti-apoptosis proruin). Follicular (B-ccll) I ~ m p l ~ o m a are . ~pIenornttgaly.! ic marker) : tlyp. H T C T patients h a w a highcr incidence of 11011-Hodqkin'5 lylnphoina. (~t~ell-dirferentiatecl) or diffuse-9pu (poorly clifikr-entjared) ly mphocctic Iymphoma. md plat elel. i ncrpawcl Feruln n n d urine uric aoicl Ir. i s c o s i ~i:t nlay he primill-y (pnlvcyiliemia vera) or q~cnnrS.A 62-year-old~lrwisli male visits his fxrnilv doclor because o f epistaxis. Phlebotomy: hylrnxvllrea. to a Irssei. Hc alcn drscri hes episndes or severe generalized itching ( I V ~ urr U crs) . 011esi 0..) . hemoglobin ievel.. . Pc.r. and hematocrit: C1.. in ~rlegakaryocytes and WBC precursors: t h r o ~ ~ ~ h forrnacion us with microinfi~rcts ill brain and hrart.o m l > i rol-mation ~~ n~ainly in licart alld h r a i ~ i )sttbr~orrr~al . Eieadaclie. PE VS:hypertenqion ( I%P1 70/ 100). Gross Pathology Micro Pathology Bone Iniwrow I)inpsv SIIOM'S increase in erythroid series precursors i11lr1. or acutc myulogrnou~ luu kenria. myelr~lihr~osi. Normal Pn?.l i q rharilcirrijled hv Atlas Link * POLYCYTHEMIA V E R A (PCV) .Z) "months ago and M a s pi-rviuu. smoking..o. exreti t.114<1 increawd. IeveIs: increased leukocyte alkali~~e p hnsp hatafe.t=lq:demaqed erythmpoietin level ( c l i s t i r ~ ~ i i ~ l l c s pulvcvthcmia vcrit from sccunciary p o l ~ ( ~ y h e ~ n i i ~ ) .d frequrn? af peptic 111c~f. and dizziness. The p a t i ~ n c hnd black.. PC\' may pl-ng1-erqr o chi-onic n l v e l o g c i ~ o ~ ~ s Icu kcmia.u-y(due r a COPD.TRC. lnav t-nsue with cllaracteristic Iindings. Labs CBC: markedly increased RBC count. myclofihrosis.r~aved viramin F5.. primarily after showering. PE: ahese and plethoric.V. plrltplpt !'unction (bleeding tcnrlcncy) : increast. Increased blood volume and viscosity (RBC s l u d e g and t h l . tortuous retinal w i n s with dark rerl hue on ( i i i ~ d ~ ~ ~palpable c ~ p y : spleen.. Polyc! 111~1ni. mild cy~nc~sis: cngoraud.. treat Treatment Discussion I~!-per~~riccrnia ittl increase i 11 FSC: mass \zrith incrrasecl bloocl vul~~rnc and r .1tir~ii.lv admitted tu thr Ilr)spi la1 L'ni deep venous thrombosis. i n ~ d P-T: i~lc. tarry stools {VT:LI. eic. 12habrlan~vosarcorl~as m e aften found in "cancer families'' (e. grape-like mass arising from wall of vagina. eosinophilic cytoplasm ancl cross-striations. SARCOMA BOTRYOIIIES .ID/CC A 4vear-nld fernale i s t>i-o(~ghl t>y her mother tn the pediamic cl i nit afrer qlip (iti~ls blood and a "lump" i n the child's vagina. 0 z ( 7 0 G 1 2 o m < 2 ". Sur. polypoid. Bulkv himor mass with mu1tilohecl papillary prqjections resembling mass of q a p e s . The child's fathcr died of brain cancur. Gross Pathology Micro Pathology Rinpw of trltnor tnasl. Li-Fra~~rriei~i 5yndr.ancl stool yields tlo pathologic findings. arid her rnother i s rccriw ng treamwnl for b r ~ a scancer. Sarcoma hotrynidcs is a polvpoidal: subtype uf embryonal rhabdomyosarcoma that characteristically protrudes like a mass of' graperi fi-om the cagina or kladrier. I>luocl.:i t i s [he mosl coin m o n sarcoma in children.g. i Hrt. HPI PE Labs Routinc Iah u-ark on urinc.orne).granrlfarhrr d i d of rneiastaiic col(lr. Pelvic w a r n reveals ulcerated. elnngated rhabdomyohlasts with largr. s l ~ c > desrnin~ ~ s and myoglobin-positive (muscle ttlmor) . T Treatment 2 3 r o Discussion < ---..gical rrscc ti011 ~ i t :jc!jlivar~t h c hernother-ap): radin~hel-apy.ectal cancer. e hemoglobin eleccrnpl~oresi~ shows 85% HbS. PS: pallor: mild ictena: f~mtluscopy revealq hypoxic spots with neovascularization ( " S F . peripheral film (Sickledex prep). FIhS Corills poljmers that damagc the RBC membrane. Gl~~mtnic acid is a r h s ~tuted i bv valine nt positinn ti. CT/ US. non hcaling c hrorlic r~lccr o n left luwcr*leg. Imaging Treatment Discussion Atlas Links fl SICKLE CELL A N E M I A .ocal ~ h e r a p v for leg ulcer: laser tlierapv for proliferative rctinopathy. i n : autosomalrecessive inheritance. Labs CBC/PES: rlecreasecl hematocrit. HoweM-Jolly bodies and Cabot ring. sickling o f R&Cs on sodimn metakis~ilfitt. megalohlastic anemia: sickleshaped N C s . Serwrn bilirubin rnoderalply eletraied:q1iantirath. Fatturs that hastun sic kling i n ~ l ~ t d acirlosi.A 10-year-old black child prewntq with a c l ~ r n n i c nonhel c e r otl hi5 lower leg. calcified spleen. leading to chronic henlolytic ancmia.i r and hyoxemia. abdon~un: srndl. Sickle celI anemia i s rai~serl by a point mutation on the gene totIing for the P chain uf l ~ c m r ~ ~ l u li~ ~show?. His tnar ernal cousin s~lffct-s Ti-om a hloort disorder. VS: fcvcr. LIA: micsos~opic htmaturia. Prcni~taI diagnosis is ax..rilal~le for at-risk fctl~scs. hydroqwrea may heIp irlcrcase lktal hemoglobin levelq. In the reduced form. I. anti biotic prophylaxis against capsulated bacresia. He hac had recurrent episodes of abdominal and chest pain (due LC) micl-ovascttlar ocrl~ision) along with diminution of vision. \ F:ws'') . Labs Imaging Gross Pathology Micro Pathology R ~ r marrow l incrcascd. TIIC child'^ parents are Indian immigrant<. yeIlow 1nar1-owd e c r e a ~ ~ marked d: erytl~roid hyerplasia in marrow (ineffective erythropoiosis) Rlood kanqfusion. folic acid supplement.splicii~g or translation or mRNA. hlphx-thalasscmia results frorn decreased synthesis of a-glohin cIlaii~s clue to delrtior~ oi'onp or rnore or the four rw genes that are notmally present. XR. iron chelation therapy with desferriosamine to reverse hemosirlernsis.HbA absent. mildly increaserl unmnjupated bilirubin. Expansion of hcmatvpoietic hone nlarrow. hypnchrcrmic anemia with anisopoikilocytosis. 0 0 Treatment Discus5ion < ^ I THALASSEMIA-BETA . and bone marrow transplantation using HLi-matched sibling dnnc~rr. skull (lalural): maxillary cwrrgrowth and wider~ing of diploic spaces with "hair on end" appearance o f ff-nntal hone.K ~ C I E S: ~splenomegdy. dccrpaqed 1-erirulocvtosis. HbF 95%. mild ictcrus. and delayed developmental motor milestones. failure to thrive. causing thillrling of cortic-dl bborle or new bone formation. HPI PE Marked pallor. ) CBC: severc microcvtic. Beta-thalasscmia resulh from cl~cr-easecl svntl~esis of fl-globin chaitiu rltw to errors in the ixanscription.IDJCC An I I-month+ld malc presents with ntarked pallor. frontill bossing and maxillary hypertrophy ( ' ' ( : ~ I I P ~ I L ~F N. causecl hv vertical trabeculac. low platelet munt (50.2) wit11 s~r king i retinhocytosk ai~cl fragmented RBCs (sr. Elevated indirrct I~iliruhin(3. shortness of breath. hemolysis). PF.~~sromms) .A "Lyearold white female cliagnosed 2 years ago as HIV positive i s 1>1. AZsu known as Musc hcowil/'s yi~drorne.000): negative Coninhs' test. which was ab~tndan anrl ~ had lasted [or 7 rtavs. brain.lacdc TMP-SMX 3 weeks ago.: pale skin and mlrrous membranes. PE I T S :ti~chvcardia: fever.enals. hcadachr.1>11ght to the Plnrrgency mom hy her husband becausc of tachycardia. intermittent disorientation. petechiae on chest and exrr~lni t ips. Shc had started propht. normal coagulation tests. Plasrnapheresis and fresh fro7en plasma e ~ r h a n g eprednisone. she had finished her rnenqtrual period. and pancreas. Absent haptoglobin (due lo in~ravascc~lar. and heart most aflectrd by thrombosis. confusion ant1 apathy with lucid periods. arll. renal cortex. Multiplc h y l i n r thrombi iri brain.at-sd aphasia. Gross Pathology Micro Pathology Treatment Discussion THROMBOTIC THROMBOCYTOPENIC PURPURA . elevated LDH. Her hnrband also points our a generalized red rash all over her bodv. LJA: hrmatutia. rnyr>cardil~m. Labs CBC/PBS: rnicroangiopathic hemolytic anemia {Hh 7. On thc previous day. r hromhotic tllroml~ocytopellit purpum ( n P ) is an idiopathic disease Found in pregnant and W-positive pal ien ts anrl Ihllowing c~posurr to drug5 s i ~ as h antibiotics and estrogens. pnri tive Bahinski's sign. Tllrnmh~ts formation i11 scveral o r p n s with platelet rlepletitln and rr~ici-oangiopathic hetnnlytic anemia: kiclney.5). splenectomy. . eaction )~~ may he the requlc of cemplete complemt=ntactivation: most c o ~ ~ ~ m o it n lis ya result of mismatched blood. ~ rushccl ~ 5 to the emcrgcncy room. hydrccorlisnne: alkalinizt. :\I] WPI He was invoIved in a rnc. vomiting. where he received five units of blood before heir18 taken tn the O R for repair of a ruphircrl spleen and liver. Treatment Discussion ~ZCLIIPl l e r n ~ l ~tir ci ~ n $ h i s i ~ t. no hepatosplenorne~alv or lymphadenopathy. decreased serum haptoglobin: elevated inrlircct bilircil~in. urine wit11 HCO. producing intravasclar hemolysis.>torrycle accident and 1 . cola-cnIored urine (due to hen~oglr)hi t~uria). rorlal shuidnwn or disseminated intrawscular coagiilarion (DICE TRANSFU5ION REACTION-ACUTE HEMOLYTIC . I t severe.gicxl lapai-otomy worirld unr-emarkable. sut.. Hyclratiorr: tbr-ce diuresis with martrlito! or r~~roscmide. PS: Labs Positive Coombs' tcst (indicating a ~toan r til~odies to RBCs) . and chest pain fuIlowir~g i\ I~lotld transfi~sion .ID/tC 18-year-old hospitalized inale cornplains of fever. nausea. PE VS: fever. or. G'S: !'ever. rrspiraturv dis~r-rc. icier-(1s. HP lias resrived several transfusions in the p m . An hnun. RP nol-tnal: t a c l ~ y a r d i i i ..IO/CC During the administration of a blood transfusion.)rcsorits zvi t l l fever. headache. . * TRANSFUSION REACTION-FEBRILE NONHEMOLYTIC .hiii~.IS a few weeks ago.. all of wh ich were unevcntf~~l. 1 1 0 c~anosis. Thc last oiit W. Suppc~rtirc. and facial flushing. Skin rash anrl pl-11r-itns or anapliylaui5 occur it1 rrllel. HPI PE Labs sel-11m hiliruhir~: no CRC/PBS: negative direct and indirect Coonlbs' test.gic r~acrirrns media'ted by IgE (due to n type I hypersensitivity reaction).larer h p cI~vt=lnps frank rigors.: ~nai-krrl ~>alloi. PF. a '$5-ymr-uld male I. Normal incon~patibititr' fi~i~n oli d reprat czuss~nnlcliing ol'donor ~ r u m and patienl's hlood.lndi~qion rmction ic ratrqerl hy preformed l e n k o a g u (qwttosirantil~orlicq)dt-i)elop~rl af~elP~PV~~PIS lri~tlufl~\ir)ils it : i s prinririlv : i type J J hypersensitivity reaction.In tipvrrtics: Ie~~kocyte-depIete ruture transfusions hy Liltration. r~irlerzt. Treatment Discussion Frhrile nor1hemolviic t~-.: facial flii. Tympes I and J I are autosomd dominant. PE Labs Pmlongd bleeding time. nlorlerately prolonged PTT. lo~v \MIF acl hlty Dcsmoprcssin.He]. quantitative xsay for factor VlII reduced.~ci(n(vMT) arltiget~ levels. She has a history of prolonged bIeeding fc~llowirlg rr~iriii~~al Li+auma. cpisraxis. Mucosal pctcchinc. avoid aspirin. low von Willcl~mi~d'r L. virallv . platclctc do not axkTcratc. with ri~toceti~l trst.~ttrn~~atccI v11T conccntrale (Tfl~maie-P).platclet Treatment Discussion I 52 0 r 0 3 < 0 m r-l z P a C1 < * VON WILLEBRANDFS DISEASE . von IVillrhrai~d'~ discasc is also callcd ~ a s c u l a r hcmopl~ilia.ID/CC HPI A 12-vear-uTd while female is 11rc)l~gI11 trl thp emergency room k~ecausr of uncontrollable bleeding following a tooth extraction.father also ha5 a bleeding disorder. R c-ommon congellital disorder of hcmostasis. vWF factor is necessary fol. Waldenstrbm's macroglohulinrmia is a m x l i p a n t Blympl-tt~cytc diqorder chal-acterizrd by excessive XgM (rrracroglohulin) production ancl hyperviscosity syndrome. Treatment Discussion Fla. and visual disturbances over the past sevcl-al:monrhs. PE Genrralized lpphadenopath~ engorgement of retinal veins with hemorrhages: r~loclerate tiepatosplenomegalp CBC/PBS: anemia (Hb 7.ID/CC A 68-year-old ~vl~ite male visits his doctor complaining of weight loss.mapheresis: chlol-a~nbucil. . Imaging Micro Pathology XR.. I. increa~ing fati*~e weakness. RBC rouleau Formation.te\).3).iopw may be labeled pleornoz-phicIvmphorna. plain: absence of lytic lesions (vq. L!mph node 1. multiple myeloma). Imne marrow and spleen typically infiltrated with plasma cell precnrsar5 (plasmacytic lvmphoq. mav show cytnplxxmic cosinophilic. PM-posi tive inclusion bodies D L:T<:I rER RODIES) . IgM paraprotein (n~or~oclonal spike on serum protcin pier t mpliol-esis). headache. cyclophrlrphamid~.lr also complain^ of eay bruiskg and bleeding gums while HPI I~rushing his teeth. . increased Labs sel-tim visco<i~y. U A : normal. mild splenomegaly and cervical Ivrnphadenoparhy. Labs CBC/PBS: thrombocytopenia:lvmphopenia. A male cousin sul'ress Crnm a similar illness. The conditiot~ is associated wirh an increased incidence of lymphornaq. capnilar.IDJCC A 2-yea]-+Id male i% brought to liir pediatrician hecause of rccurrent epistaxis artrl chronic eczematous dermatitis.. ccxmatous dermatitis ovcr 1~0th legs. Treatment Discussion Largely supportive. bone marrow transplant. and increased IgA.polysaccharides of bacteria). several purpuric patches over skin. Decrexqed isohemagglutinins.and T-cell deficiency chal-actcrized Ily a triad of tharnboqtopenia. and recurrent pyogenic infections. PE Epistaxis.e. eczema. inability ta form XgM antibody to carbohydrate antigens (i. f 3 0 < m ' 3 0 Z WISKOTT-ALDRICH SYNDROME . HPI H e has a history of recurring pneumonia and hilateral chronic s~ppi~rnrive otitis media. decreased IgM: increased IgE. ilorrnal IgG. it i q due to a delelion of ihe WASP gene in the p l l region of the X chrnrnosotne. splenectom): L Wiskoi t-Aldrich synrlrorn~ is a rare X-linked recessive diseaqe with B. edema or t h c ankles and knees. SER'UM SICKNESS . splcnomegaly. the child rlevelnpcd fever. and Ict1iat. Ai~tihistamines:cor~ico~tt~-r~ids: aspirin. VS: li-vex-. Hrr.IDJCC 1 1 &y~ar-oldwhite female is brought to the etnergency mom hy her mother becalrse n i severe itching.p. She had received an injection of penicillin 6 dayr3 before for stre~~ococcal tonsiEIiCi5.axillar y arid inp~inal l~mpl~adcnnpatlly. C4 levelq. rednrss a11cI swelling or knees ancl ankles. ORCC in the hospital. Treatment Discussion Scrurn s i c k n e ~ is s a type III hypersensitivity reaction (irnmurle cnrnpl~x rt isease) with a Iat~ncy period I~etween exposure lo the d e n d i n g agcnt { d r ~ l gserum) ~. Vascular lesions show fibrinoid necrosiq and a ner~tropl~ilic infiltrate: immune complex deposition i n kidney andjoints. PE: gcn erali zerl urticaria1 skin rash. and a generalized skin eruption. including allergies. cpinephrinc if srvel-e. Gross Pathology Micro Pathology Gcnrralized wheals throughoul hnrly. and lhe appearance o f s i p s and s y p l o m s : it is usrlally sellllimiting. hcmaturia. UR: proteinuria. HPI RE Labs Jncrea~ed ESR: decreased GS. hematuria. joint pain.mother denies any reIemnt past meclical history. A 50-year-old male presrnts with complaints of palpitations and chest pain. 4 u s n ~ Fr . resulting in niyocardial ischemia. Imaging CXR: "tree bark" calcification of asce~iding aorta ail$ arch of aorta. VDRL and FTA-ABS positive. comperlsatory irregular fihroits thickening or aortic i~ltima. U'eakening of the aortic wall cansrs clilalat i o n or the aortic root ac weIl as aortic incompctrr~ac arid aneurysms. gradc IT1 early diastolic murmur heard rddiatir~g do\vn right sternal edge {rn~~rrnur of aortic inco~nprrence). Treatment Discussion Atlas Link " SYPHILIS-TERTIARY (AORTITIS) . Inti~rlalfiln-04s causcs nan-owins oi' the openings of the coronary arteries {ostial stenosi5). PenicilIin.Echo: aortic incompetence. d~generation and til~rosisof' outer two-thirds of aortic media. mid-dias~olic mtlrrnul heard at apes ( .mediasltinal widening and cardiomega?iy.H A M M F RPULSE) .hvpertrophy and dilatation. Labs ECG: left veniric~dar hypertrophy with strain pattern. cardio~negalv: loud aortic component of S2. wide pulse pressure. Gt-nss cardiac hypcrtrrlphy (cm. collapsing puke ( W ~ T E R . hortitis occurs in the tertiary stage o f syphilis.INI. Ute pair) increases with physical activity and is relicved by rest.M I~ R M U R ) . left vcntsiculal. VS: high-volume. surgical excisinn and rcpai~. He has multiple s e x d partners.olving tlxe arch and thc ascending aorta and rxtenciing into the sol-I ic valve.bovinum) . aoriic aneurysm Gross Pathology in\. rendering it incompetunt. orten arising many rlrrarles d t e r thc primary inrection. PE: p15toI shots Ileal-rl over braclliaJ artery: to-and-frv murmur hearcl over fernoral artery (Drr~nzrb'/'s hi^-RML~R) . Micro Pathology Obliterative endarteritis of viira vasortrrn. all MI-negative i~lothers with an MI-positive fetus shur~ld receive RhO (D) immune globulin following deliveries. T h e mother did not receive any subsequent immunizations.erlrR1k isoitnmun isatiotl.Ts). increaser1 indirecc bilirubin. cyanosis. ectopic pregnancies. antibodies ow in^ to her exposure rn D antigen during hcr dcLirery of an Rh-positive i n b n t . d w n e a . Pallor: marked jaundice. Brain spccir~lun from au tops): reveals yellow ~taining of hasal ganglia by ~inconj~rgated bilirubin (IU:IINI(XEH~~S). t Iiese an tihodies crossrcF the plac~nta and reacted with the fehlq's RBCs (Rh positive). h epatosplenom ~g~ly. prodl~cing hemolysis and fetal heart failwe with g e n d i z e d edema ( H ~ K O P I+:TA~. I-lcr previous childbirth w a s an uneventf~ll full-term vagirlal delivery cnnrll~ct~rl nutsidp the United States 4 years ago. exchange transfusion. PE Labs Gross Pathology Treatment Discussion The mother prodt~ced anti-ll (I$) ERYTH ROB LASTOSIS FETALIS . generalized edema. Nenna te's serum: poqirive direct Cooinha' test. HPI Her mother is blood type AB rCh-negative. blood type of first child A TUI positive. and marked jaundice. Phototherapy (promotrs elimination of hilil-ubin). Mot her'q serum: positive indirect Cwombs' test. hypotonia. anti-D antihndv titer > P:64. S T c 1 pwxre. BEaocI 6-pc of mother Rh negative: bfootl tvpe of-faliler A Rh posirivu. abortions. SS and S4.IDjCC A terni funale n e ~ h o r n is notccl to have edema. o r even amr~iocentesis. In her sl~hseqzlen t pregnancy. and scattcrcd foci of Iymphoqtic tissue in interstitiurn nClungs. the mother admits to being a smoker and remembers that the child had a URI 4 days ago. HPI The child was siight3y prernatllre. Autopy reveals pctechiae on pleural and poricardial surfacrs. it is clear that tlie child has heen dear1 ror at least 4 hours.sc 2-month-old male.~lleda1 7:00 a. his history was unremarkable. PE No pathologic causc rr-vealed that could explain death. but aside from this. most have a history of mirlor UFUs. canilot he awakened by his rnoil~er: upon arrival. Gross Pathology Discussion Sudden infant death syndrome (SlnS) refers to death of an infant under I year of age. h e c a ~ ~ a thc child of Cuban in~mfgrants. pulmonary congen ion. in which death remains unexplained wen after co1np1eteautnpsv. On directed history.m. IDJCC Paramedics arc c. SUDDEN INFANT DEATH SYNDROME (SIDS) . usually during sleep. These was nothing that c o ~ ~directly ld explain t h e episode. oliguria: I~ematllria. azoternia with BL'N/creatinir~eratio of 3 (within t~nrrnallimitr). PE Labs Corlfi~wd but alert: underweight. PG-P2-048 f reatment Discussion Atlas Links I k - ACUTE TUBULAR NECROSIS (ATN) - . elevated urinary sodium (> 4 0 tnErl/L). and weight loss. Kidi~cvs enlarged. Discontinue offending agent. HPI TIIPpatien 1 had bren o n aminoglycosides. flahky. ancl pale cclc~nii. Gross Pathology Micru Pathology Necrmis of t111~1lar rpizhelial cells (hat 5lough into lutnen. a-'El PM-P2-048. Increased serum inorga~licphosphor-us. Acll te t 11bular necr-osis i s defined as acutc tu bulacr darnage resulting in acute renal failure: it is causer1 bv prnlongerl iqcllemia or toxi~ls(ncphrotoxic i l r ~ ~ g iind s ) is usiially se~bersible. fluid and electrolytr management. forming casls aizrl causing hlockarle: hydropic ~ I e g ~ n ~ t a t i of on epirl~elium. I l l i e n questioned.yt PS: it~creased p o t a s ~ i l l tUh: ~ ~ . renal k~hular epithelial ccIls in scdirncnt. I. h11t despite his c o n f ~ ~ s i o hc n clescrihe5 llis l~ritie as appearing rerlclish+range over the past CPW weeks. nausea. he is ~ i n c ~ r t a i of n place and time. mild pmteinul-ia: g1-annlar casts in tlrine. isotonic urine osmolality.IDJCC A 1 7-vrar-olrl whire male undergoing c hernotherapy foldissprninated 1-lodgkin'sIvmphoma complains of serrrc hcarlacher. nc) aci~te distress. In autosomal-dominant cliscasc causcd by :I dcfecl in chromosome 16 in which the renal pa~-c~~cf~ is y cor~~er.mal parenrhvma: cvsts thick-~t..>: wi tli hunclrcds of cysts hat almovt replace nor. Gross Pathology Micro Pathology Treatment Dialy~is ancl rcurtl n-a~lsplaiitation.d herry aneurysm. C:v?tic dilatation of tubt~les. which were attril~utccftu an enlarqerl prosrate glar~rl. DpC" ADULT POLYCYSTIC KIDNEY D I S E A S E (APKD) --- - - - . nruru: ruptu1. Kidney tnarkecllv c ~ ~ l a r g e. His father died of chronic renal failure. ranging froin it f e tnillilnefers ~ to srveral cer~tirneterq in diameter. a n d his p. Ad it1 t polycvrr ir kidnes disease (.D) is . sp1ee1. ' 110 ) .: palpable.It is associated with 1w-t-y anrurwms of the circle of M'iIlis. PF. crcatinir~c.tetl m. VS: h~pertension(BP 170." He also descl-ihw ~low-onset dull p i n in his 1eTr flank and bloorl in hic urinc. epithelial cell hyperplasia: c ~ ~ b o i d a l epi~ helium lining cyst<.4PF. livcr. l ~ n ~ ganrl s . Cmtq ITl.c~ lnd hein.CT/LlS.e. non tender abclomind mass n r I t ~ cth flanks. SIP1 PE Labs Imaging hngio.t In hllnrlt-erlsof fl~~icl-filled cvsts. F-IU was recer~tlvtr-eatect for recurrent UTIs. resulting ill p n s ~ e s i v u renal f~ill~re in adulthoocl. and niit~aI yaIw Discussion .ID/CC A 4 7-yar-old whitc male enters the emcrgcrlcy r oorn cnt~zplaining of :I sudden-nn~e~.duo involve the panclras. severe headache that is the "worst headache of his life. rr1icrc)rcnpir hemahu-ta ( n o MTRCs or casts). hypertension.lternal grandfather rlicd of cerebral hemorrhap. al~dntnen: multiple kidney and liver cyst%. Sfigf~~lv increaser1 BUN.lV . nuc ha1 rigidity CA: alhuminuria.1.allrd. It is also . procli~cing cha~acteristic laminated appearance with glornvl-r~lar wlero~is. ~ e A VC:EI. bilateral cataracts. interstitial infiltrate containing fat-filled rnarrnphages ( 1 ~ ~ rF. Treatment Discussion ACE i n l ~ i b i l o rrenal ~. Her mother surfers frnm chronic renal fbilur-e./PBS: nnrmoc hromic. UA: proteinuria.v r a ~ n l d to the pediatrician because her !nother tlnticecl blood i n her urine and diminished vision acuity. normoqtic anemia.female is brought ID/CC A 5 . HPI Her family is Mormon. transplantation Alpart's ?mrlrnme can he autosomal-domi narlt car x-lit~kerl: and i s caused hy a defcct in tho or chain of tvpe n ? ' col2ayen. 1:s: BP normal. High-ton? ren~orineuralloss detected on audiomctry: eletpated serum cre. PE: appears well nourished: bilntcral sensorineural hearing loss.uiiiinc and RUN. RI3C casts. Gross Pathology Micro Pathology Lorigjtudinal thinning ancl splitting r ~glomer~~tar f hascrncnt mern brane.s1tloot31kidney.LS). Srnal't. PE Labs CIIC. hematuria. ll tiqsue. Her past medical history is unremarkable. Imaging Gross Pathology CXR: biventricular cardiac enlargerr~ent. Treatment Discussion Primary amyloidosis cnmlnnnlv prescn ts ~vivjlhnep hroric synclrornc. a1 Ins. amyloirl depositinn in mesaugizim a5 well as in er~dotheliun~ surrounding hepat ir si t~i~soids and in spleen. Micro Pathology Applegreen birefringence in polarized light when stained with Congo red. ascites.Feyear-olrlwhite female complains of palpilacinns and shortilcss of breath. hmyloidosiq mnv hc primary (in which the proteins arc monoclonal immunoglobulin light chain) or secondary LO ch t-rmic inflammatory stales (esperiallv rheumatoid ariht-itis a11d tubel-culc~sir). pa): and firm: spleen and liver ]nay be enlarged. waxy. The primary typc is ufien associated with &cell clyqc~aqias. primarily heart. and in lhcse ca5es Bence Jones proteins are altnost alwavs present in the serum ancl mine. cardiac arrhythmia on ausrulration. morning s\velling of the eyeq. Mi lcl cardiomegal:?: rnacroglnssia. especially multiple myeloma. Atlas Link . and nt~mbiless of thc lower legs together with weight loss and Catigue. hyperlipidemia. clecp-brown discolnration characteristic of amyloid-infiltrated organs exposer1 to iodine. Labs LJ-k proteinl~ria. ECG: ~ ~ e n t r i c ~ t hvpertrophy lar ar~d Icw vnltagc (resttictive cadiomyopathy). Supportive.A 4. and rertal ancl gingit. Pathologic deposition of amyloicl glycnprotrin irl several organs. pitting edema in lowcr extremities. Hymproteinemia. Elvaline thickening of arteriolx watlq. kidney. leading LO narrowing o f lulnetl a n d ischemia. kidneys palc. arlcl legs. He also atlmits to liavitlg a reduced caliber of urine stream ancl terminal dribhhg a? well as urinary hesitancy.~ir~s of haling night 10 urinate ( ~ n ~ m : ~ i .calerl ~ i~ctd\iles 1111 1 0 1 crii in diaiii~telin median lobe pl-mtate.ge-rtlprnszvle 1t. positivtz nitrite and leukocyte cqtcrasc.rrcir>oma).r~staiicr. P~. is notcd starting at 40 YtzillASof age. \ ) .*ndr. Imaging Gross Pathology En lar..r t r n tion rmc n a o r l ~ hago that was i*rlie\. Atlas Link BENIGN PROSTATIC HYPERTROPHY (BPH) .~si~rd pr. PE Dipj.nrlent changes o l ' cststjgr~lu and .rtlrvit11 cathe1~1-iyation. Micro Pathology Treatment Discussion A~t:c.~r. P-S: hrnign-appraring enlargernrii t of' nirdian I o h ~ .i1 1 wcll-de~n.t (w.ltcdSCI-urn crcatiilinc :tr~clRP:h'.ng~nr arc. Tt ~~ffrcctq up t o 75% o f ~ n r n Ilv lllc a g r o f 8 0 vrars. Tlic patirnt's histnrv iilcluclcr onc episodc of acutc UI-iriary I .nst. Iwlie~lerl tr. catlsc hen igtl pl-nutatic tlvpcrtl-ophy ( BPI-I): :In increasing incidrnct. bladder percussibfe trp to ~unbiIicus. Hr denim any historr nFhc.lr~at~~ri.-old male corn plains nl' urinary frequency anrl in~~rruptio of n thc urinary sb-rarrl owr tllch pa51 fi tt~nrllh~: lie wake up mt~ltipFe rime5 cluring the ~rlso compl. Both strrlma ancl glandu shc31c' hyperplasia on 11ir)psv: lih1-c)t1tvc~atlei10ttia1n~1~ perpla'iia 5ee11 i n which ptnIiCet-ating gla11ds arc s ~ ~ r r n n n d e by d prolifrrating ~ m o o t l i rn~lsclc cells and fihrt>l~laut~.-rlcpr. rnctast.iie-hprcific anligen (PSA) lrvrlg nnrinal: ~ ~ r r ~ r l y n a n i i r sntrlie5 rlert-tonqtnt~ bladder neck obsimction with incrcasrd reqirlual urine volume. ur~rcinorrsa of the hli~dder) or I~nck pain ( r s . A 5fiyear. Labs UA: 2+ bacteria. milrlly cIc~.ltal rrctal csam rcr~rals smooth enlargement of the prostate pl-otr-m-lingi r ~ t o the wcri~til: ovetlving rectal muccjsa mol~ilc. piihelil~rn and projecting inlo blatlder. hone. Imaging Gross Pathology Pc'odular. LTA: hemat~lria and a l x u ~ ~ l a ppit11eIi. C. nt. analgesic abrse (especially phenaceti~t ) . IX'l-'/Cysrojiram: irregular filling defects above trignne. tlle lung.d nt cclls. and li~~er. i ~ i ( F 9q.A 65-year-old white male cnmplnins o f painless hemahria OF several &T-s' rl~~t-alicl~~.~l failt~i-e) . invasion w-i t l i 111-inaryol. palpable masses. siagt. Complirat ions i ~ c l u c l e invasiori ot per. Sclr istosoma haerna tobitinl infPcti011 (although innst Srlrbturomn infections are associated wirh Fquamtnls ~iroptasis). Micro Pathology Treatment Discussion Thrrr i\a t hl-eeCr)lrlincl-casc iri r-i\li i l l men. ar~cllonglerrn cyclophosphamide I l~erapy. Risk fz~ctors f o papillary ~ rxrcirloma of the I>l.ytnIn~. cigarette smoke.rr~ocytic. abdurnen nnntencler. I.~clrler inclrtde incll~stsi-a1 expnsllw L n arylamines (especially 2-nap11t hr lalninr) .C) a arising fi-orn arr)c. anrl m r castases tr. Labs CBC: slight nor. Biopg' nl' hladrler shc~ws p p d e I.ivesicrtlar tisst~c. g c n i ~ ~ l i a wi~llin rlortnal limits: n o Iy~~~pl~arlenopatl~y.rv of ltri1-1~ sl~c~ws rnal ignailt cclls. cauliflower-like lcsior~ ~ vlh i cell tral necrosis ar~tl ~r~iiiirnal inva~inno f hladdcr wall.11ng~ clcar. R transitional cell c ~ o m ('1'C. He i s a heavy smoker. nnrrnucl~rolnicanemia.s~ruction(luxling Ijycll-nnej~ pvelnnephl-iti~. Atlas Link -T BLADDER CANCER . ancl rcn. TGC apprars 10 IIP asrnciaterl with rnuta~ions ill rile p53 h ~ m o suppr-~fsor r gene anrl clclrtion\ in chromosomes 9 p . nnd thc average age ai cliagnosis i< (ij. ureteral h~osis. ~r~cmia. dccseased forcc a n d stream uf ilrinc.~litir. kidncys-s: biIateral hydroureter and hydmnephrosis. lae has notril m increased frequency of mictut-itinn along w t h inct-ea~ecl hesitancy. 7 BLADDER OUTLET OBSTRUCTION. BUN and creatinine. Labs CRC: rlr>tmocv~ic . L p s : hyncalcemia. Treatment Transrlrethral resection of' I he pr'ostiilr (TURP) r o 1.q)and in irtdividuals older hat1 ti0 vearx. NEPHROPATHY . h y l l e r p h n ~ ~ > h i ~ ~ u Elevated ~ i i a .ulcl a furling of hcompTete evacuation of thc I>ladcIrr. urgency. PE Pallnl-: hladder fill1 T ~ ahrlo~ninal I exan~ination.~1ieve the r>hslrl~ctioti is the hxsic atld most 11sefi11 SIPP. US. Cll-~strurtive ncphropatltv is common in chilcI31ood (from congrni tal ahnorrn. w h e n benign prosta~ic hypertrophy and prmtatic and gynrcoEogic cancers become Inore common. recta! exam reveals grade K D prostate enlargement. For lhe pxqt few yal-s.FOFthe past fcw months h e has h ~ g t n t o rxprricncr increasing fatigability and laqstude. 'Discus~ion Ol~stt-wtivr nephrnpattlv rcsz~lts from t h r impaired orttfiow o f urine hut rrlay 31x0 produce chronic interstitial damage. In aclrli tion tn h~dl-otwphrosis and livrll-o~~rctcr. t!A: pmteirl~~ria. >l. interstitial Imaging Micro Pathology kidney di~eaqe is w e n on m icrnqcopic exxmination.IO/CC HPL A 65-vual--ol(t rrlale prrsen ts with acute urinary retention. no KBCs or seen. PT normal: rici ther kidnt.5 g m / P 4 hr).1 mg/dL). ~m ISI 11. PE \IS: hypertenqion (BP 160/ 110).llinc arrcriosclcrosis of both affcrrnt and effrrunt arterioles: 110 i i i i ~ n u CCIIII~IPX ~~e depopi~q qew.tl biopw: thickening of capillary basement m e m h m e cnr~~hinerl with acellula~e( )ritiophilir n o r l l l l ~ in ~ i n e s a ~ i g i ~ (KMMI. Elevated fasting blood st~gar (23.ihle: F I I I I C ~ ~ I F C C ) ~ ~ C exair) reveals prcsence ol' proliferative diahctic rctinopathy. PE: gep.y or tvpe 1 diabetes mcllitus hut no history 01' Ilet~~atul-ia.0 5 5 D I A B E T I C NEPHROPATHY . avoiclancc or ncphrotoxic drrigs. UA: P I . n o cvirlcncc of pleul-al c f f ~ ~ s i o or n awites. ~levnttd ql~xnti iative pl-otei11 (3.ld white fefurnalc il. Treatment Wscussion Dinhrtic glon~crulosclernuis is a t-cnitl n1a11ilesiatinn o f diahe~ ic ~nicrnanginl~atl~v atlrl presenf? at l e a ~ t 10 vears aftet-diahere~ appears (mol-ccommonlv in IDDM). I . dialysis ur rcnal o-anspIantation. I t u ~ g I. rere11I sol-r tllmaf or skin i t-tiertinns. Labs Micro Pathology lncrezed rnesangial matrix cln srn. Atlas Link mP M .~ 2 . decreased serum albumin. acl~nitrrcl to the hospitill l ~ e c a t ~ s e of worsening generalized edema a11cF rveakxlesl.~ W ' T I of L-~ stigar and S+ protein: bvoa~1 C ~ S I ant1 S fatty casts.~ran auscultation.ene&ed pitting edema. dielary protein . pl-efcriiblg tvi th ACE irlh il>itor.ID/CC -4 4%vean.ase% clr. control of svstcmic hypci-tension.W I L DIS~CLTE): ~~N hv. it is ustiallv thc prcludt. elevated blood chfialesterol. along wir h hypertension. . ele\b. HPZ She has a l o n ~ h isr01. .~tirl phnsphare yes! tictioll.i~cd glvcosvtared I~cmuglobin( I 0 % ): elevated BUN and serum creatinine.): palp. . Rloorl sugar control. to end-slage dial~rt ic renal rliseaue. Ile rlcnic~ nlcohol t l s r hu~ arliiii~s 10I>eiilg a lieaw smoker.111enl mcmhr.spy. I I)$: r l l i q i z r i : ~ :hmaturia: pro teinuriz.I C ' I tivi I S ~ I? rt'ac1io11) . * GOODPASTURE'S SYNDROME . L)il'fusr p111monn1-vcrncktrs hi1:ltcrallv. anti-glomerular 'basement membrane antibodies in ~ n .~ ~ ~ ! i~ifilt~:~~es. ncc~~mpanicd by liyl~rl~carlotl~it. gTgfomcruloncphritiswith a r r ~ u n ~ ~ l a tor i n ~ieutrophils u and rn:~crnphaqe~ irl Blxvrnan''. ar~emix c : ~ r ~ ~17v ccl anti-. immunos~~pprrssivt. Discussion C:ontllx~st I Ire's ~vnrlrotr~e i~ heinorr3i:rgic al~erll i ti5 with ncphri ti\ and iron rleficienn.n <lcsc~-ilws in~errnittei~t I p w r AIICI tit'acIaches in arlcIitivn r o small vc3l11ni~s 01' dark orange mine. ~Eier.~lon~crr~lar bi1st. HPI He . and ninlnise: yrstpl-day lir co-righedup blood..hot em ia. C ~ I ~ S Ichal-3ctcrisric I~C: linear T g G deposits in glomerdar hacement membrane and alveolar septa on i1nm1111oll~1nl-e~cc~1re.~asc ill iwigh~ ol' I~tng-5 wit11 areas of r~ecrosis: kidneys enlarged and pxlc with clecrcased crsnrirtcncy Kiclt i ~ hioprv v st~ows pl-r>liI'el-. PE Labs Imaging CXR: l ~ i l i l ~ talvt-oi. Incl.u r nnccrc~rizi~ig : hcrnorrhagic alveolitis on lt111g Gross Pathotogy Micro Pathology i>i<I]>5yn Treatment I ' l a ~ n i aexchange: corticoctet-oids. tI3Gs: hvpoxcrriiit.ID/CC 11 S&year+ld ~ v h i t male r cornplains of a chrwnic c o t ~ g h of'slrl~eral mot1thc' dt~ration. nWI'f1 t i ~ i n gcrescentic .mc a11t i l ~ r its ~d ( R ~ C I1 ~ V P L ' I .11.r tiire.~r . f311 i g IP.rs. . Iron deficiency anemia: hloorl d ~ t c c t e c in i cputurn. it iq especiallr: u s c f ~ ~ inl imrlrl-lying i~chenlic hexrr discasc. i t ir. HPI He a l ~ o reports markedly diminished urine output over the past 24 hours.i the cafest ancl most efcctive drug for usc in hypvrtcnsive errirrgencies: I3ecarl~c it docs not irl~pair mvuca~-dial bloclrl flnw. Treatment Reduction o f diastolic blood presstlre to at least 100 rnmHg. VS: severe hypertension. Elevated BUN and creatinine.*). abdomen: presence of parenchymal renal disease in norrnal+ized kidneys (unlike that of henign I I P ~ E I I-OSCI~I-osis. . hc .ilso reports some vis~ra'l blurring. On direc trcl que~t ioi~ing.INNIN~. inerrtl~olizrclto c y r i i c i t ' xnd thio~!a~lale. maintain urine output > 20 rnL/horw. Imaging Micro %thology PatIloIogic changrs includc fibrinoid necrosis of arterioles ( N I:< ROTl71VC 4RTERlr31 m s ) hyperplastic arteriolosclemsis ( " 0 ~ 1 0 SU. ~ and n e c r o l i z i ~ glotner~llitis ~~ associa~ed with a thrombotic rnici-crangiopa~hy. ~vlvheretllcr-c are I>ilatrt-a1 contracted kirlnevc). Howrvet. CBC:: micronngiopathic l~enlnlptic ailcmii~. Blood tlli4 ~ cana y re Ievcls should be cteterminert frequentlv.eciureri ejectinn fraction. proloilgcd us(: rnnily lead LO r!anide toxicity or to thiocyanate tnxicitv. Sodirun nitmprusside i. FE: flrndurcopv re~~eals presence of papiIIederna with hypertensive retinopathy. Discussion . ECG: lefl-axis rlrviation with Ieft h m e rtticl~lall hyertrophy Echo: ronccntric left vrn~ricl~lalhypertrophv tvi~11i. thrt-ef'ore. PE Labs ViZ: protcinuria: microscopic hematllria: red cell cmtq.EDJCC A 45-vual-uldblack male prescnts with ~ ~ r ~ c o n t r n lhypertension led and complains ol' severu occipi~al hearlache ancl ringing ill h i 4 ears. LTS. slight palpebrnt cdci-na. Increased serum IgA. Pallor. The glomerular pathology seer1 in B~rger's disease is similar lo that wen in Henoch-Schiinlein purpura.itivu. ~vliich is seen in children.ZI: ~rrnteinllria. TgG. gross henlaillria.in patients with t r l i a c disease and liver disuase (due rIefectivc Ig14uImr. or possil>lyct-ewendc glomerularlephritis. HPI PE Labs Hc was well lrt~tilthe onset of'wtnplnmq. Lt.ID/CC . red cell casts in urine. It is scrn with illcreased f.aiice). IgI:4 ncpkxopa~hy is idiopathic hut associated with rlppcr respil-atrlr y cji. Discussion . postrtreprococcal glomcrulonrphritis).I 2-vear-old white male romplaills of rec~zrser~l epi~nrles of "bloody h e ' ' that laslril Tor several clays in conjunction with a URI. Focal glotneri~lnnel~hritis involvirlg otjlv sclected glc3meruli wit11 mesandal proliferation and segn~eiltalnecrosis with crescents: immunofluorcscheucerypically reveals mcsarigial TgA deposits with w m c IKIW. Chronic rend failure may ultimately develop.cqlre~lc!.4orls are variable and may he mesangicprolifurdtive. IgA clep~sits r o r n r ~ l ~ l treappear ll~ fvllowi tlg kidney I ransplantation.G T infections lacking a laserlcy period (VF. focal ptnliSe~. and C3. Micro Pathology Treatment S~ppol-t ive. Zr. '" Cni. i t iliclt~ces severe protrin~rria (11. weight loss. Tl1ei. 1 1 0 r ~ t l o c h r o ~ nanemia. !. HPI She alqn has a history of recurrent oral ~~lcemtions a13d a phoiosemihe skin rash. oral aphthous ulcers uoted.transplant. nephritis. Class I11 is chamtesizccl hv focal proliferative glomc*rl~lo~~el~h an f 'd i~ is is assnciatccl wit11 r-eclu-renr l a c n ~ a h ~ r i a arlcl tnilrl r e n d i~ls~rfficiency.oirls.joint deformities art: reported.~!I ~ i o l ~ reveal5 w 1eatu1-csof diffuse proliferative glomerulonephricis. La bii C. n o ahdominaI o r renal I>ruitq hrarrl. Elevated RUN and creatinine.nephrntic qndrunrc=.ticost~r. cry lhernatnus photosensitive skin rash: "butterfly raqh" nver 1 1 1alar area of hce.e are five pattcrns of I i~pu. as rnesangial lupus gZomeruloncphritis and is foun rl in ahout 25% ofpatients.ID/CC Pc 30-year-old black woman preqcnts with pain in both her knee joints and in t l ~ e srn.BC:: normocyt is.Claw I S ' is clcscril>eclin tllis rase and is hv far thu rrlosl common I'orrn. antinuclear antibodies positive in high titel-: LE cell phenomenon posi~ive.1ll-jnint5oCthr h a n d logether wit11 milt1 fever. ic FA: ~nicroscopic hemau~ria zvi t h RaC casts in aclclitit. n d c11Ior. i t is .~nlbucil). Rcn. EM. iuathiop r i n ~a . Klertmn microscop\ reve. and i~nmt~nofluoresccncc luicroscopy. atwl-&a. Class V pre5ent5 as membranous glomerulonephritis ancl i'i seen in 15% or cases. anti-Sm antibody and anti& DNA anFibody positive.. PE VS: h y p e r ~ e ~ ~ s i o PE: i i . cyrotoxic ill-11gs(cyrlopho~pharniclc.n to pl-oteiuuria. Inng-term hcrnodialysis nl.lls immune complex deposit9 that are typicall! subendothelid nnd form "'wire loops. Class I i s ~iorrnal Ily light. Micro Pathology Treatment Discusslion LUPUS NEPHRITIS .isw~ciaterl with minimal Ile~natitria or prnteinuria.No. pallor. and 1099 or hair. Clam I1 pl-ewnt c. VDRL positive but FTA-JIBS negative. Micro Pathology Treatment Discussion Ven~hnnopr. neither.per~~ension ( HP 140/ 1 00). normal A S 0 titcrs.~l.. C a r e f ~ que4tionin~ ~l reveals that shr has a h had hematuria.lthwav). cheri palpitalions. and ringing in llcr ears t o ~ e t h e r ~i2h generalized edema. It is subdivided into two I Y ~ P S : npe I MPGN (hot11 classic a n d alternatir~c complerncr~t pathways activated) ancl type I1 MPGK (clcnsr deposit diseasr. renal transplan~a tiori .ophy.~l ~rat~rplantation.IDJCC AIT1 I-year-rllcltvlii~e girl is brought to the perliatrician hucausr of hc:tdaclic. liidnpy palpnhle. Dii'fusc glornc. anrirlt~clear atltihorly (ANA) negative.-11 arid sohenrI(>t Eirlial tlepositr of inin~iine cornpleseh. Cort icosteruids. She 11x7 nn hiqtorv nF dvspnea. no P V ~ E ~ E ' I I Cor P pleu tal eIr115iorlor ascitcs. otlcvrr.mrs. MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS (MPGN) .I ~tion of nltrrn. SpIitting of has em en^ membrane causing railroad-back appearance with ! ' A S reagent or silver ~ t . lung hases clcar.t tcd wl-iim ~righcerides.lr involve~rlrnt with ~hickened capillary walls and FohuIar ~ n u .oliferxtiv~ glr~tner~alonephritis (MPGK) is idiopathic hut mav he aqsnciated ~vitliiuflcrited dcficicncic. ~ i prominent n: granular immanofluorescence. PE:: g e n e d i e d (including prriorl~ital) pitting ederna.tngi. a c t i .~tecomplcmrn t p. Fore throat. Thrr-c is a high rrpcurrence rate Lollrwing rer~. HPI PE S'S: t~\. w ~ ~ gprcdiferatic~n iaI or) lighr rnictnscopv. skin infec~ innlq.K will go on In rlev~lop chronic renal failure. UA: fatty casts and oval bodies i n addition to proteins. Labs Elrva~etl BUK and serum crei~~init~c: clecreased s~t-uni alh~~min: clcv. of conlple~nent compo~lrn ts arld partial lipudvs~r.JrP nnrn~. Apprn~iniatelv 50% of psticnrs w i ~ h 3ZPC.r~rl. serum hypocomplementemia. pitting erlen~a in exmerniaics. ~ . Pallor.: wi~h nephtncic wndt-rrme have.lntat inr1. hpercoaplabiity sccondnrv to locs of antithrombin I11 in t h e trine ( p . Gross Pathology renal t~cin thronlbosiq may Micro Pathology Thickened basement membrane.) . Labs UA: proteinuria (> 3 . clecrmsorl lung sounds with cracklcs hilatcriill~ in Ic~wcr lr~ng Iirlcls: periorbital edma. lipiduna with o'i~:~l fat hodie5 a n d Iatw and was? casts in lit-inary sediment. rriinimal change cfiqeasr ( I .lthc. p a k . anrl swelling of the lower leg and arrn5.ll vein thl.ss.pitheli..nmhnsir). (the m(w1 cnmmnn ca~tsc. incidence pcrip11e~-. hyperlipidemia (serum cholesterol 250 r n ~ J d L ) . in adults). Patiet~~. pl-ng~-es~ive shortnew o f 111-path. Hrr past meclical Ilistor).~l depclri ts of IgG i ~ ~ CS l d along h a ~ c m c n t nlcrnhranr srcn i1-r "spike and dome" pattern un m c t h c ~ l a m i n e silver qrain.. .E~I-IKO~ (the I \ ) most common in cl~ildrrr~).. mites.inrreaspd . yclophosphamiclc. and be p ~ e s e r ~ t . Discussion " MEMBRANOUS GLOMERULONEPHRITIS (MPCN) . I I Y ) I I V ) I. ACE inhibirors redricu urinary prmteiri Ir. renal transp1. Treatment Corticr~stcrnlrls. IbcA glomer~l toscler(3~is. or mcmhranoproliferalive glorner~~loiiephri~i~. 1-11bherv. Hypoallbumhernia (< 3 g /dl. Iiidilevs r tllargecl. h'cphrotic m~cIrumc may hc idic~p~thic or cauqetl Ijy m e n i b ~ a nous gl:lonrcr~ilr~i~rphriti. 5 g/24 hr). is tiii~-en~ar-kal~Ie. i m m u n e cleposits in a "lumpv-bumpy" (disconrinurrr~s) pattern o n ~~nrnunofl~!crr~ercrtice.h 47-war-old black diahetic Ternale complains ofwcight luss. EM rcrcals uniform and diffuse l n s s or the poclocytic foot processes. normal serum crcatii~ine and BVN. It carries a good prognosis. HPI T h c child had a URZ 1 u ~ r k agn.I t.ID/CC i2 5year-olcl white rnale presents \v<tl? generaked edema and ahdnminal clistcntion . producing respiratorv embarrassment. Hypoalbuminemia.I I ~ I ic111ic calcium: normal CS levcls. Discussion Xlsn caller1 Lipoid nephrosis. nlinimal change disease is tlze most common r m s c of idi oIjarf~ic nephrotic r. frce asdtic fluid in prrironeal ca~ity. hypercholesterol~mia: hvpet-triglvccriclenlia: rlrcreasccl s e i . I . PE VS: 13P normal. liid~lt>\~s slight2y enlai-zed. salr-restricted dict: dilirei icq: eIccnoIyte therapy and rnonitclr-ing. Atlas Links fIFZI PM-P2-062 I IM2-036 . soft. shifiing dullncs~ and fluid thrill present. nortnal rund~~scopic exam.yndrome in children ancl is associate(1witla ini'cctiuns or vaccinations. PE: generalized pitting edema. Labs Gross Pathology Micro Pathology Treatment Corticosteroids.igl~r micrascopv and immunofluorescen t shldies normal on renal biopsy ( n a ~\<rLcnce of immune complex deposition). proteinuria ( > 3 g/24 1). L J k . ancl vcllowi5h. and hack pain 11ia t has lasted several months. Imaging Gross Pathology Irregularly enlarged. firm. irrep4ar area of indrrratinn irl peripheral lobe of prostate on d i ~ i t arectal l rxilm: midline fwrrow between prosta~ic lobes obscured. sacrum. forming osteohlastic lesions.3ttncnt depend heavilv on stage.A tj8-vear-old black male complains nf dysuriia. Labs Markedly elevated prostatespecific antigen (PSA) and acid phosphatase. prostate: hypoechoic masses in periplleral zone with extensioi~tn seminal vesicles.prostatc carcinoma is thc most common male cancer. Thr tumor can a l ~ o invade sacral nerve rOOF& causing significant pain. extension to serninal vesicles detected. r o c k . NIIC. C T / M R: prostare mass with capqular pener ration ancl ~tilarged seminal vesicIes. Its prognoqis ancl rrr. Most case5 are diagnosed in asymptomatic mcn un digital rectal exam. pr~~gressively increased u h a r y Frequency.trlt. Transrectal VS.h d . and pelvic bones (axial skeleton). A primary malignant neoplasn-i o f the prastare commonly arising from the peripheral zonc (70%).rloplastic struma. IJe report5 high animal-fat intake. Inosr row mon lv to bone. Micro Pathology Gore needle biopsy of prostate reveals single layer of malig-nant neoplastic cells arranged haphxardly in . Prostale cancer exh i hi ts hernatogenous dissemination. nodular prosla ie. Treatment Proqtatectorny with radiation. or-chicctomy: leupralide: antiandrogens such a s flutamirle. brine scan: hot lesions of spine. Discussion Atlas Links . Nodulal. cunsidcr renal-sparing partial ncphrcctomy. Yellowi~h areas of necrolic [issue with fncal areas of hemorrhage within rcnal parenchyma. n T J C T J U S :mass in upper pole of right kidney. renal cell carcinoma is Crcqticntl~ speradic hut is. Atlas Link PM-P2-064 h RENAL CELL CARCINOMA . Labs Imaging Gross Pathology Micro Pathobogy PoIy~onalclear cells ( c r l t ~ l atling i glycogen) with evidence of rytologic sepia invading renal parenchyma. normochromic anemia. PE VS: To. Right nephrectomy. PE: pallor: pdpable mass in right flank. and renin). Treatment Discwssion . seen in association with von Hippelk d a u syndrome and djalysisrelated acquired p o l p t i c kidney disease. The most common rend tumor. ACTH. moderaw hypertrnsion. CRC/PBS: norrnoqtic. Elcvatcd ESR. MPI H e Elas heen n heavy smoker for the past 24 years: he lost 5 pounds over the past month and i s net on a diet.ID/CC X tifl-year-old white inale complains of r i g h ~ flank pain and hcmaturia.~v:r-ade fever. t'A: gross hemamria. I 1 rreqr~enOy invades the renal vein and TVC arid mctastasi7es to lungs and hone ~ i Iletnatoget~ou~ a dissemination. Ic can also cause paraneoplastic syndromes (secondary to the pr-c3rI11rt ion r )f erythrnpoietin para thyroid-like hormone. MR: no invasion uf renal vcin o r infcrior vena cava (RTC). is a hcar?? smoker and drinker. Crwnplic:\tion. nausea. Labs an zrrior Normal RUN and creatininr. FCC. Gross Pathology Pale. yellouish-white. atid 1-tas Iwrn surgicallv treated for aortofernoral occluskve disease grid^). a1. RENAL INFARCTION . Treatment Remove arterial obstr~~crion IIV thromholvsis. swc-ating.~guI.. has !>ernrlial~~tic T o r 15 years. hvpel-rensiotr. wedge-sl~apecl area wit11 hernorrllagic necrosis in renal cortex. xanthelasma in hot11 PE r-vclirls. st-vtre right flank p. infuctious enrtocarditiq ~egetatinnq. PE: acute fislress: pilllor. UA: hernaturia. -dial Elevated LDH. US. HBf He is overweight.domr.n: wedgeshaped. Iwparin an tico~gulation ro prevent recurrence. mild hvpcrtension (BP 150/10O).: old silent ~'ilI~ l ~~yo~il~ i~ifarctiotl.~in. and vomiting. Micro Pathology Cr.fr-urn I-rrlalartery rmhnliqtn incll~rle renal failure. Imaging CT. acute pycIonephritis. and rcnal abscess. VS: nn fever.ttion necrosis irlvolving renal cortical nephrons cxtcncling into cortitomed~~lla~junction. Discussion Risk Fitcror~ Inr emholic events inclr~dc atherosclerosis and r n ~ ~ rthrombi al in the Iieart and aorta. nonenhancing lesion in right kidney. and atheromatous plaqiics in the atma.ID/CC A 63-!car-old white male compIains of sudden-onset pain in the right flank lugether tvirll gross hernaturia. rei~al: edeinatous kidney with Focal region nf dpcreaserl color flow. ~with fibrosis and segrnetltal s!enosis. Gross Pathology In fihromu5cular dysplasia. the rei-ial artery lumen is decreased dur.enin ti-om Fiypnperfuwtl kidiwy(s). I t is mast ohen cauwd by fibromuscular dysplasia (young Caucasian women) or atherosclemsis (older men) ancl accountq for < .u. Mi~scular hygerplasi. ~uhvpcrpfastic fibrotic wall thicken in^. Micru Pathology Treatment ACE inhib'itors (contraindicated in hilateral renal artury stenosis).ID/CI: .I~ypertcnsionis secondarv wsteinic hyp-pertension rai 1xe~1 11)' I~y~el-secret ion of r. cof all caucc-s of hpc-rrrnsic~n. s z e n ~ n g~unjcal . Ik~lloon a t~ginpla. unilateral lcft renal artery stenusis i n a "string of pearls" pattern. 4 30-year-old &it? physical vxam.itv. cnniirmator~. PE Labs Imaging A n ~ i orcnal: . Atlas Link T 1 PG-PZ-066 RENOVASCULAR HYPERTENSION . female iq fmind tn be hypertensive on rolltine HPI S h r claims lo have no history of hypertension 3 r d denies any changes in lifestyIe or excessive strcss. Elevated plasma renin: hvpokale~nia. PE: loud S2. hlnrlt~rcopicexam normal.5%. abdamind bruit present. VS: hypertension (13P 1 75/ 105). Discussi~n Renovascul. corrrction. S&yeardd wliitc male prcscnts wit11 progressive painless enlargement of the left testicle of' 2 mnr-frhs' rltlration.ci. Seminnma i q tlre most cornmon Cpc tjf germ cell rumor.I good pl-ngnosis. s111uot11.~ nf p a i n or ir-auma a t the ~ i t e . Dvsgpl-lninnmaq in nvarie~ are histnlngicnlly similai: T11rnni-s iivp extrcmc-IT radiosensitive.~ris. Walni~r-~ized. tabs h'cjrrnal lcvels or hC:G. normal levels of serum a-fetoprotcin and LDH. Cryptorchidism prcdi5poscs 'to the decelupmcn~ of lev ic~llar tltrnol-~. histologic ( i i i t q n m i s hased o n postopurative specimen stuclv.lst. chcm orhcrapv ni th cispl:~tir~. firm mass at upper end of left testicIe: mass does not bansilluminate: epididymis and vas clererens normal on palpation: prostate a n d srminal vesicle5 t~ol-inal oil digital rectal w a r n : xhdnlninal lvlnph nocles not pnlpahlu: no hepa~ornegza2v. Imaging CSR: n o met. He also cumplains of a w i s e or hrsviness in his scr-oturn. Discussion Atlas Links .~ted rvi th .s. Treatment OrcIiicctomv ui th rctrc~prrilonralItmph nocle diswcrion.US. He t l r i i i r s i i r l y histnl. rilciiot het-apv. nontender. ahdonlurl and pelvis/scrotum: 5olirl intratc5ricular m a w CT: no 1neti1st. Grass PathoIogy Micro Pathology Shepts of germ ceIls ctin~aintng clear cyoplaxm wi rh lymphocytes in f i b r o l ~ s strclma.tsi. It is assr. ir~g the right testicle. ( t h t~~ i ~ ~ ii~ o removecl. Polvgunal. Treatment Chemotherapy with cisplatin. scrotum: complex. and blromycin in qome cornhin. PE: bilateral gyneco~nastia(breast tissue paIpahlc) . PE \rS: normal. I Labs CRC. mixed with multin~~cleate syncytiotrophoblasticcells that have eoslnopMc vacuolated cytoplasm with readily d e r n ~ ~ bhCG. I Discussion TTESTICULAR C H O R I O C A R C I N O M A . Serum 0-hCG elevated. gynecomastia rrgres3es once rhc source nf hCC.Fnllowecl lrry radical inguinal orchiectomy ancl retroperitoneal lymph node dissection.: mild anemia. Imaging Gross Pathology Small. testicular sensation lost: no Iransillurninatiot~. l e no well- Micro Pathology dcreIoped villi seen. left s~~praclavicular lymphadenopathy. and hlnorl-streakccl sptl~um. h epa!nmeply. srnaI1. CXR: I I ~ O''cannonball" parenchynlal masses (due to metastases). r) Choriocarcinoma is thr most malignant of all ~ ~ s t i c u l a turnurq: r i t metaqtasizcs relarively early via bolh the fymphatics and she bloodstream even w h e n i t remains very small locally. co~nparatively r~nifclrln cytotrophoblastic cells with clear cytoptasm growing in s h ~ e and ~ s corrls. aabclornrn: erilarged rernvptritonral lvrnph riodes and multiple hepatic nletastasw. pea-shaped swelling involr.ID/CC A 30-yrar-old man cornplaiils of a small painless nodular swelling ever his right testicle that he noticrd a few mo11~hq ago. HPI He also compIains of mild rhortncss of breath on exerrion (nmpu~x) cough. . p~a-shaped henrorthqic mass seeti in right testicle. Follow up with P-h CG levels.~tion. US. solid r i ~ h resticular t mass. etoposide. T=T. coupled wit11 increaqing growth of his breast tissue. si rticrl tres Imaging and presence of dysgenic Micro Pathotogy Testes rh:1rilr1~l-i7erl hv seminiferous tubule degeneration and i~~vasion hy connuctivc tissr~carranged in wberlx. the mother did not take hormones or. hormone replacement ~lier. I Ir9 TESTICULAR PYSGENESIS .I'O/Ct A nelvhol-n hahy is uv.bilateral inpina1 swelling. Discussion T ~ intitlcnce C u F gonadal tumors in d~genetic gonads rnav reach 1123 tn SOW. Miillerian structures absent: inguinal swelling4 prmed lo he maldescended dysgenetic testes. US: absence of miillesian trstes. i n a k i n ~ ot-chirttr>myand s ~ ~ h w q u e n t hormn~le r s p l a r e r n ~ttthe ~ hest tl~erapeutic option.~py given at puhertv.duatt.cl for ambiguous external pnitalia. The baby was delivered v. HPI PE Labs Ki~i-vurttw: 46.iginally a1 fill1 term without any pw-.XY. any o~held r u g during p r e p n c y . Incompletely virilized external genitalia: Ii!~ospadia5. Treatment Conadectomy to protect againsr increaqecl risk n T testicular tumor. posrnar~! complications. or. inrra-. wlier~aq rhoriocarcirlotnas procluce oi1Iy hCG. It i s thr most coinmon neoplasm in men aged 20 to 35. left supraclalicular I y n p h a c l ~ n o p ~ t lk ~ ym . HPI H P denies anv history uf STDs. Imaging Micro Pathology Treatment High I-adicalingttinal orchicctomyt foIlowerl by cisplatin-based con1billation chemotherapy. US/MR.Wk sac ~~~~~~s ptnduce 011ly AFP. Testirulitl-cancer rnav he pure or niixerl (mixed germ celI neoplasm) and is higl~ly ~~lalig~nant with early xnrt widespread metastaris. bilateral enlargement of the breasts (I:~~KC:OMASTL~).1nal rectal exam. or Il'illltllit.ID/CC A ?hear-nld white male is seen I)? his Faillilr. PE Bilatcral nonterlder gyiiecornastia (duc iucreaserl hCG). drug use. hard mass palpablc on righi testis. Discussion " TESTICVLAR TERATOMA (MIXED) . Cvtotl-ophehlastic and syncytintrophohlastic ceIls with h C G dcmonstralhe within cytoplasm. no1.physician because or dmnea.ites: solid inmtesticular mass with some Coci of hemorrhage (iiltratessicr~lar masses usually ~r~aligrlan t). genital ulcers. Labs Markedly elevated blood hCG and a-fetoprotein ( A m ) . arid a painless lump in the right testis of approximalely 2 monchs' di~ration. 1e. distorted shape. l~iseniecl ar~rl vomited three times. swollen.~zed TI-oin orcl~iti~. I r i s seen more Frequentlv in an ondescended testide (( ~wroirt:i-rinrs~).ID/CC A 9-yeat~)lcl ldack male is hrougt~t in to the errlrrgrncy room hecause of sudden-onset severe pain that he expericncud in the lower at>domen and scrotum while p l a ~ i n g soccer.righl testicle tender. and e1cv.x):propliylaci ically (high incirlenre or bilatcrality) . Treatment Immediate surgery (clttclrsion . increased pain with elevation of mass (PRI.ltur. Nirc-Tc9S: doughnut s i p (due to ccntr.~lflow).md fixation of tesris to scmtum) dnc to risk of iusiicle loss (less than 4 hours). he llecame n. in tel. Discussion Tcrticular torsion is a s l ~ r ~ i c ernel-genet. scrcllrtiii: aqvinrri~trirrlecreaserl color flow Imaging Gross Pathotoqy Tcsticlr rnarkcdlv cnl. I IV'S SI(:N). atrophic testicle should hc rcmu~ccI due tr) puwihlr autnimmune rlestrucl ion of' con il-alaterxl testis.t~-gcd will1 hrmtlrrf~agic necrosis. palpable HPI PE r~orrnal epididvmis antcriorlv. al 1ha1 needs to hr clirk~-enti. Labs Uh: mild le~tkocytn~is. and s t r a n p ~ l a t ~ d I-lertlia. r p o n admission.ll lec~ic~tliirischemia ancl circl~mferential co1l. Tsl-itakility.ttcd. contr-alatcral orc11iopc. 1 1 0 her-nia palpalde: 1 1 0 transfIlurnination nl tnass. scrotlrm may bc p ~ i ~ p l i scol-rl h .ll history. TESTICULAR TORSION . epidirlymitis. Micro Pathology Severe venous congestion : interstitial hemorrhage: hernorrl~agic necrosis.zicle. U S . twisted. He has no relevant medit. and pretreatment with allop~~rinol are keys to preventi13nof this syndrome.ind hyperphosphatemia with scco~idxy hypocalcemia. and p hosphatc may restdl in precipitation c ~ these f subsratlres in the kidney Renal sludging and acutc renal insufficienry or failrire Flirt1 1 ~ 1 aggravate . PE Carpoperlal spasm prcsunt. fIuid and clecuoly~e balance must bc maintained m i d clialvsis may be necessal-): Tumui. or preexisting renal insufficienq~ increases the likelihood that a. IrA: acidic urine with numerous rhomboid crystals: no casts or cells wen.lysis syndronle is tnost often seen in patierits wit t i lymphoma or leukemia hut is also scen in patients with a. and fatigue :$ti hours foIlowing chemotherapy eeament. once acute renal failure has developed. b~isk alkaline diuscsis. BUN and matinine eIevated. an increased pretreatment LDH level and uric acid level. tlle Discussion F?a URATE NEPHROPATHY . i large turnor burden. mriety of solid tumors. The presencc uf .purkalemia. Incrrased Ic-vels o f uric acid.hyperuricemia. . xanthinc. Labs Lytes: h?. patient will develop tumor Iysis uyt~drotne.is empty. n e i ~ h e r kidnev is palpable: urinary hladdel. a high growth fraction. severe malaise.ID/CC A 45j-ear-old man with a high-grade nan-Hodgkin's lpmphoma develops olipiria. Treatment Main tcnance of good bydriltivn. he involvement o f perirenal fat. genitor~rinary al~normali tics. ~ n d smooth intl-a-ahdoininal mass to right of midline: sight cryptorchidism and aniridia. hT:displacement and distortion af r i g h ~ pelvicaliceal sysrem. WAGR syndrome consists of Wilms' tumor. metastasis 11sualIyto lunp.i r ~ v a q i o ~ ~ . persistent ellipsoid arm ofenhancemen t (dile t o compressed renal par~nrhynla) . UA: ~nicroscopic hemaiwia. may. 11 is associated with deletions on chromosome I lp involving the WT-1 gene and should he diffcrcntiated from ~ ~ e u r o h l a ~ t oa rn d a malignant Ivmphomn. and mental retardatinn. BUN increased: serum crythwpoiedn clcvated. or striated muscle tissue. solid rumor wit11 awaq nF hemorrhagic ~lucrosis distorting normal renaI parenchyna comprew~d inlo narrow rim. fii-m. Slight pallor: weight and hcigh. Treatment Discussion WILMS' TUMOR . radiorhelapv. nontcnder. Ncphroblastoma (WILMS' TL~STOR) is a malignat~c tumor of embryonal origin. Labs Imaging CT. Gross Pathotogy Whitish. normal llrinarv vanillylmandelic acid (VM. Micro Pathology Glomcruloid and t~thulnr structure5 enclosed within spindlr cell stroma. HPI PE The chiId has heen well . within normal range. .4).A 3yearald male is brought to his pediatrician for cvalualjon of an abdominal maw that his parents noticed. of kidnev con raining tnmnr: chemotl~erapv Surgical rcmov~l with actinomvcin D and vincristine.ill his life. aniridia. which are other small cell turrlnrs of childhood. areas of carrifage. large. bone. no widence of vasculal.abdomen: mmnr at-isirrg from right kidney with areas of low densirv (due to necrosis) . H P I She had 11nde1-gone a cholcn~stectorny 2 d. r~arcot ic overdose. Treatment . m d n e a r . dissemina~ed in travascu1. hagtnented TU3Cs: thrornbocytopenia.jurv. hyaIi t ~ e memhrane formation.I t i~ characterized 1 3 ccliCF~rw ~ alveolar capillary in. i t is caused by gram-negative sepsis.IQ/CC A 4. she vomit.ar-old whitc remale is r r ~ s l ~ u to d the OR because of shock rlne 50 postoperative hlccding. a n tihiotics.5-vc. P E central cyannsis. heal? lungs which. PE 1 ' s : tachycardia: tachypnea. and inflammatory infiltrate: loss of surfactant with fihsohlasr activity i n later 5tages.issociatecl with high mortality. during in tl~hatiorl. Labs CRC/T)BS: marked leukocytosis with neuh-ophilia.~lr. produce stiff lung with fiht-045.eolocapillary clilmagr rvirh edema. Gross Pathology Micro Pathology 1711dr )t I-rrIial and .lr coaguIat inn ( DIC) .Mecha~iicalven tila~ion. close monitoring of I l e m o d ~ ~ a r nfi~nction. Fomatioll of hyaline membranes wirh proteinaceous deposits in i~lveoli: pulmonary edema will] red. Imaging CXR: typical diffuse and symmetric parahilar ("hat-wing" pattern) alveolar fitling process suggestive of noncardiogenic pulmonary edema. moist skin: intercostal retraction. hypotension.r l r w n i t ~ g . acute pancreat i ti?. comhirter? 1vit11 widespread atelectasis. and aspirates thar day7shreilkfast. ic Discussion Adult respirarorv diqtl-ess synrtrome is a corldition that i q .burns.~vsbefore atid had presrn tcd with postoperative blecding requi~ing surgical explorr~tio~~. ~ I ..~qerl131TN and ct-eatinine. Atlas Link ? ADULT RESPIRATORY DISTRESS SYNDROME (ARDS) . warm. fever. steroids. i ncr-eas~rl AST and ALT. Inrl-e. inspiratory crepitant rales heard over both lung fields. ABGs: severe hypoxemia with no improvement on 100% oxygen.t i c h Icads t o a n incrcasc in vascular pcx-mcitbilivand p~ltt~ot~a eld-e yt r ~ a . mawive trauma. 1~ L OO-pack-year smoking historv. T C HP I auscu1t:rtt. * ASBESTOSIS . We i~ a retired construction worker anrl has a nca1.r lo1)es wi 111 cirr l t tnscri hed racliopaqiae cl~nsitirs( ~ ' i . .it. lirtear. Treatment Suppnl-tive anrl syrnptnt~~at ic treanncn t (oxycn.d ~ PE VS: normal. This process is seify pc-rpetuating. interstitial infiltrates in 1owc.c to asbestos in ~igt~ificar~ tlv curnulatir~ close. Grass Pathology Micro Pathology Calci~iinconraining dmsc plruraI opaciries ancl plaqltes of cnll. PE: pxtle TI dubbing.M i ~ t . antihiocics):prevention of h t h e r exposlue: smoking cessation: counseling re~ardir~g high risk orbranchogenic carcinoma and malignant mesotheliorna. I~~-onchodilators. cor pulmonale.lge~i . PIT<:niixetl ohrtrllctivc anrl restrictive diseiise patt ~ r t l reT111ced : Dl+tl. malignant rnesothelioma. Imaging CXR: ir~reglilar. Discussion Prolonged rxposr1r.. 1x11cessaiior) 01 eupuullr may slow rliseastl prcgre~sion . CT (high resolutia~l): posterior anrl Iatcral p l c ~ m thickened ~ with calcifiecl plaques sren bilaterally. Cnmplica tion5 itlcl~idebranchogenic carcinoma. arxl death. lirte c ~ a c k l e hilateri~lly over lung bascs. Labs 5 0 z CRC normal. Difti~se pulrl~or~ary interstitial fibrosis with bilateral pleural calcification and ~ l ~ i c k ieng n and i~~volvcmcn I c ~ thu f rliaphraatn. ~ u ~PL~QLIES) .+cer>t~q rnaterinl. results in pulmonary parenchymal scarring.s onnres) composerl of asbcstor G~PI-F coated wit11 an iron-con raining protei~l.n s ~ ocxam p i ~ t ~ sputtlrr~ f re\+ealsgoldenbrown beaded rods (~szsn~srt.ID/CC A 65-year-eld male prrsents with pmgreasively increasing c011gh and dyspnea r)n exel-tion. askesrc~q l~nrlies. tot-vof moderate intermittent dpspnea that is exacerbated by exercise. accessory m d e use during hreathi ng. a n d mucus. 01-al. Labs .h n ~ g y a n d pale nawl mucosa. Imaging a hypesinflation with flattened d i a p h r a p s (incrcasecl residual vrdurne rlnr to air trapping).I-LFM)FN I : R w ~ . PE: inspiratory and expiratory wheezes (due to hronchoconstric t i m . C GT~SS Pathology Hyperinflation with air trapping in alveoli. ~ L: .o m o l y : ralirlltkast. Bronchial asthma is characterized hy hyperreactivity of the airwa~ and ohstrr~ction due to hronchospas~n. steroirls: r l . She also c-omplai~~s of a hil. I t is also knowm as reactive airway disease.ID/CC A 10-year-nlcl girl is brought illto the ER in acute respiratory distress.~NN'S SPIRALS) .RP: nor~nal. The patient is known to 11r allergic to cats and pollen: her rnotller stales that she had a recent URT. and parenteral hronchndila~or~.tir~ly cosinophilic: plugging of airways with thickened mum5 (CL~RSC:HM. edema. m. Treatment Tnl~aled. CBC: eosinophilia (1 3 % ) . I~ypertraphv of rnllcolls ~ ~ a n d s : ~longatert rhomboid crysmls derived from ~ o s i n n p l ~rytopIasin il (CI~PX(:D. hyperplasia s) oC smooth muscle of Micro Pathalogy bronchi. plugs of inspissated mucus: erIenla of m ~ ~ c o slin a l itlg. Inilammatory i ~ i i l t r z t e or bronchial epithelium. small airway i nflammatini~) . enlarged ch esz AP diameter.PFTs: low FEV. HPI PE VS: n o fever: hchypnea (KR 322).kRC.peribrontliial ct~ffit~g.s: primarv respiratory al kalosiq (h per-uentilarion). hyperresonant to percu~sion. /FLrC. Discussion 7 ASTHMA . . IV catheter infection. and oversedatior~. scattered rales and decreased breath sounds. Other-causes of pnstopet-ative fever.sis. e atelectasis is i h e mnqr roinlnoti cause nf postaperative river.-old white male de~~elops a fever 24 holm aFter surgery.in the first 48 hours. f reatment Discussion Poxtnperat i 1 .). GBC: sl iff11 t nea trophilic leukocvtosis. ECG: sinus ~achycardia. ABGs: mild hypoxemia. BP normal. hypovon tilalion.ID/CI: HPI A 5l). ATELECTASIS-POSTOPERATIVE .yea. Chest phyqiotherap). m~tcalvtic agents. -0 PE C 0 Z 5 70 X- < Labs Imaging CXR: dense opacity in right lower lobe (collapsrd lobe) with elevillion o f right hemidiaphragm (due to volume 10s. (incentive spiromrtry): dcep in~pirations. tachycardia. alveolar collapse is produced hy occl~~sio due n to viscid secretions hvorcd by rccumhcr~q.~tionof l V linr veins: n o urinary svmptoms. no liematoma or discharge Crnm woiuld: n o inflamm. l ~ s ~ ~ a seen l l v later in the postoperative period. deep venous thrombosis. include UTI. tachypnea. R h o d and spliturn culture sterile. VS: fever. PE: no cyanr. He underwent an emcrgenq lapamtomy for n perforated peptic ulcer ~ i t i l o any ~~t intraoperativc or immcdiatc postoperative complicationq. and clrug reac~ious. wotlnd infection.n o calf tendcrncss. A 1. I-lc was d i a p o s c c l wit11 cystic fibrosis at agc 4 and !ins Iiad recurrent pulmonary infections r c q ~ ~ i r i n f rg eqr~cnt hospitalizatinns. n1ea4 es. tubelike.1-ycal+-oldmale pi-esents with complait~w of exertional dyspnea. chest: incrcasecl l~ronchc)vascular m a r k i n ~ s honeycoml) : a p ~ m ~ r a ~(dur l c e I(>PIICI-(111 ~hadow'i of dilated hrr~rlchinles). PE1: pallor and grade IJ clubbing nored: coarse crackles a ~ ~ s r ~ ~ l t river aterl 110th I11119 iielrl~. and occasional hemoppis.Pms: clecrtlascd F W . bmnchadilators. CT (high resolutioi-i).il-reversil>ledilamt ion. i ~ n d infections due to Ilm?~dr*~r~/lri / ) m / ? ti+. chronic productive cough. ~uggestive of oh~truc tive pathnlov. Dilatation of thc I~ronchialtrer Icads ro infections and to fnrillel. Under-Fving callses inclt~rle obstruction clue m tumor. S p ~ ~ t trultrlre nl reveals S ~ ~ J I ) J ? / T I ~n Irr Ir TnIr. Lot~g. (38°C). iticludc lung abscesses. Surgery may hr indicatccl lor localizccl or segmcn till hronchicctasis or when medical flel-apy hifs. foreign hodics. metastatic brain abscesses.JFC'C.~ I . z~s congenital disorders such as k t a g e n e r ' s syndrome. Labs CRC: norrnocytic. Discussion BRONCHIECTASIS . irre\~ersihlyrlilated bronchides extending to the plcrlra with loss of l n n g parenchyma. chcst: dilated bronchi0Itz-s with "'signetring" appearance ( duc ti3 adjacent hranrh of puI~nonal-7 artery). and R'lymt)rzr/priwm 111I~~~trlnr Complications i. cxpeccorsnts.RW. Imaging XR. anrl r n ~ ~ c impaction. antibiotics.~. norrnochromic anemia: low hernatoctil. a11d physical therapy l o pro~nnt Iworlr ~ hial dl-ainage.cystic fibrosis. Gross Pathology Treatment Supportive measures. loss of lung volrmle (atelectasis). arnyloidosis. and cor pulmonale. ~ a c h y c x r l i a(H R 1: 113): t a c h y n e a (RR 28). 1~'iIliam~(hr~~pl>pll S Y I ~ ~ ~ O ~ ancl I I C . 1 ' s : Ir>w%ratle fever. ~ tnpvit.1 IS. fever. lesions in vascular and extravsrsclrlar sites accompanied by intense eosinophilia: skiit biopsy nf ~ > l ~ rt-ic p ~lesions 't shnws vasculitic lesion-fil~rinoid necrt.00O/pL). numt. -' Ernaging CXR: bilateral upper and fewer lobe infiltrates i ~ t ~ noncavimtd ina nnclirles. EGC. and granular casts. PE Labs CRC:: mild at~enlia: leltkocyto5is (> 10. LTA: proteinwia: prewnct of RRCs. \ r ~ n r r m c ~ (111 ~ )exer-lion that are of r c c c n t onsrt. HPI tlc 1x1s also been hming chcst pain ( n ~ c i ~ n f i)l i .unq ~ h n w s he~nol-rhapyic infarcts secondary to thrombi in afecwrl artel-ieq. C.ivflic1l cloc?i not i~~vr)lrre Iizngsl thal is cliararterizrrl hp a triad of Fate-onset asthma. and pain in both calves ( c : r . eosinophilia (> 1000JpL). octxcional :Inenr~smsand seranrlary thmnihnses wen. PWs: FEV. monitor disuaso c o t w e l~sirlg ESR leveLs. a fluctiii~ting eosinophiIia. rvidespread wheezes hilatcrallv./WC. Gross Pathology Micro Pathology Treatment Pcednisolone effective in i n r l ~ l c 1 1 i g remiwion: cyrlophospharnide u ~ e rin l those refractory to srcroids. tltc artcrinl in tcrnal clastic lamina i~ clestrovrrd and intima and mcclia arc thickened. T. Discussion CHURG-STRAUSS SYNDROME . VS: ~ a c l i y i ~ emild a . mild hypertension (I3P 15n/ 100) (secourlal-y to renal vascular involrcment). .sis of mcdia ~ i mixttiru ~ h of irlflamtnalory cells extending along arlvetltitia. t. PE: mai-kcd respiratory diqrrew. and an extrapulmonary vaqc~ilitis. Hct < 35%: r hrnml~ocvtnsi~ ( > ?P00. g ~ wanorexia. tachycardia. Tran~bmnchial lung biopsy shows ganulomatol~s.000/pL). hll1os ra~ict reduced (obstructivepulmonary disease).and medium-vessel p u l o m a t o u s vasculitis (grouped ~ vth i pnlval-teritis nnrlaca [PAN].sc>lis purpuric lesions on feet (rI~rr to clrtancotis small vessel x~wulitis).ID/CC X 60-year-old male is rukrrud to arl i~llrrgist for late-onset asthma rhai has been unresponsive to bronchodilators and antibiotics.h~~rg-Strxuss svndrolne is an idiopathic systemic small. Elevated BUN and creatinine: P-kVCA positive. SITRCs. Hc has also . for several years. neutrophits predominant. shortness of breath. VS: fevcr. HPI The parient has a 40-pack-yearsmoking hiqtory.experienced chrnnic dyspnea on exertion. Al3Gs: decreased Po?. chronic productive cough. sqlramous metaplasia. usually in the mornings. decreased mv. PE Labs Imaging CXR: increased bmnc11ovascuIar markings in lower lung fields. Str+tnrocrix~ p n m moniw o r Hnmophib~a i?lflurmwo n Gram stain of sputum sample. 7T ' * COPD-CHRONIC BRONCHITIS ... Gross Pathology Micro Pathology Thick mucous secretion: cdema of hmnchial mrlcosa. inflammation: fibrosis. whee7es CBC: elevated IVBC cout~l(14. ~ l e m f e cPcn. Incceawd size and number of mucous gFands (Reid's index > 50). copious sputum. l PlTs: decreased vital capacity.000). and niultiple colds each winter. secondary polycythemia. and fever. - . PE: stocky build with plethora.ID/CC A 5(T-year-oldwhite male smoker presents with productive cough. ~mphvsema n~ay inyolye r he acinus arid the lohu te unifnrnilv in a pattern cal1c. scattcrccl rhonchi 1ljlater.: ir-tcreaserl r c ~ i d u a~ l ~ o l u ndecreased ~e. using accessory muscles of respiration.llly. decreased DL. 1 5 : mocIcrate rachvpnea. Pi~naci tlar enlphvqerna iq C O I I I I ~ O Iin ~ patie 11 ts with a.heart sound* heard distant hut normal. Treatment Discussion Ernpkyselna is defined as ahi~ormal pel-n~anent enlargement of t h r air spaces distal to the turmii~al hmnchiolr arcnmpanierl tllr desfruction o f the alveolar wallf.~lkalosiu. Atlas Links COPD-EMPHYSEMA . incl-c-asing shortness o f breath on exertion for t h e past few ~nonths. Centsiacinar cmphyiiema is con111ionlv fout~rl in rigzarette smokers and is rare in nonsrr~nkers:i~ is ~15ualEy mnrc rxtcnsivr and qcvcre in tlie upper lohes.. full ncss of neck veins during expiration.:IYISFA~E PATTI:RN) . tapper lobes most affected. ar~tibinticq cturing acute esacrrhations. Gross Pathology Micro Pathology Pattern of centrilohular emphysema: alveolar sepra arc visiblv clilrlinishrd in number along with increased air spaccs. and h a m r u x y g c ~ t herapv.111d11as a 40-pack-year smoking history hut 113s no hiqtory or hemoprysis or uccupa tiunal esposm-e lo inorganic or rrl-ganic dusts. brc)t3chntlilatois.1.A 55-)~eawld ninle complains or progl-essivt=l~. Ccssatiun of smoking. Air space5 dilared. /FVCratio (oncr 1r1 11. PE: moclel-ate respiratorv distress.d parmcinar. termed cell triacin. s~ptmids it1 resistant caws.. Labs XBGs: mild llr~oxia with respiratorv . chest barrel-shaped pemzssinn nole hyperresonant.-antitrypsin deficiency. PFTr.. FEV. or i t m w primariIv iilvolve t h e respiratorv hronchir~les. cardiac and Iiver duIlness are obliterated.~r. Imaging CXR (PA view) : hyperlucent lung fields with a few b~~llae: flattening of diaphragm ancl elongaied tlthular I~eat-t shadn~v. Hr x l w ~ romplains o f a nonpl-oductivc mild cough . the pelvis. CRC:/PRS: i1ii. il Intrrmittrnt positive pressure vcr~tilation wit11 190%nxxygen. marked <dv.ntn bocyrop~nia. R . u?ing accessor-v ~ ~ ~ u s r 01' l erespiration.y to oxygen thcrnpy. and the ri@t humerus. later.r. altered sensorium. PF. Obstruction of pulrnonar y vessels by far glnh~r!eq. supportivr managurncnt. rl~stained following a fall from a 20-fool-high stepladder. tend cyanasis.'*) appra~'ance nf pulmonary infiltrates withnut carcliomugaly (due tn r~oncardinge~iic pulmonary edema). chemical p~~rurnon iq. s wheezing heard over both Iirtlg Cielrls. Micro Pathology Treatment Discussion Fat cmbolizatinn usually occurs 24 to 72 hours after fractures of the shafts of the long bones. bilateral perihilar ("R.I DJCC A 37-year-old male in t h c ICU devrlops petechiae.%T-M'IKc.: delirium. HPI PE VS: rt=~~t. Fat demonstrated in urine and sputum: normal PT a n d PTT.plain: Ion%l3one rr:ici~~res. Imaging CXR: earlv. PM-P2-082 Atlas Link FAT EMBOLISM . r . normal. Labs ABGs: profound arterial hypoxemia with hypercapnia. Twenty-Fourhours a5o311u %$as aclnlittrd ro the hoqpital with fractures of the shafts of both femurs. and marked dyspnea that pl-nve rprracta1.ipnea. ell5. in which in halcd qcr-run protcir~s from pigeons or ~. Fil~rosis wit11 honeycombing. is caused by inhalation o f a thermophilic A ctisnmjlces organism present in moldy hay and grain. His synptoms a1.pjc. qlutc~xic. ~he I~II cortles inlo canracr rviih moldy hay. irnmtule cvinplr?r. Ftypei-sensitivity pneuri~urtitis (. HPI Hr also complnins of'n dry cough anrl mild fever. He does not smokc and drinks alcnhol occasionallv..in-year-nlcl farmer p r t ~ ~ e nwith t s sei7vrVshortness o f breath ( I ) W P K F .g.~ct with a ~ p e r g i l l spores.. in tile fields.r ex.. Elcvi~trdESR...rllei. c~pr~ciallv T ~ . CT: al-eas of grour~d-glass abr~orirlalities with c r n ~ r i l o b ~ ~ perihrnnchial lar nodule.a~-akt-eb ind!~re thc svi~rlmme.~lverstit is) rrfrrq In in tcrstiti~~l Iung dircnsc thnt results 1rt)m inhali~tiouc ~ organic f a~~tigrn Hyersmrilivizy s. ~~s stesoiris.IDJCC 2 1 . primarily rirppl-rc.oi--cytrhr )xic T r. Huinirlilicr luilg rtiwasc ~ .eolal-walls as well as sca ctei-cd g r a n ~ ~ l o l nwith a < Coreign body ~ i a tnccllq. ~a11d ) fatigue.c s u lii-om t ~ cxposurc to cont. p i ~ e ~ ~ r n ic q~ i ci !>clievrcf ~ir tr) h a w a n irnrri~~r~olugic hasil. Srricr ay(>idanccof cour. C)t her cornrlro 11 cauqes nl t ~ p p ~ r s e n t ivi s i ry pnrumonitis i n c l ~ ~ rpigeon le Im-errIer's rlirease anrl Iil-cl Cancier'~ clisrasc. T C tabs CEC: Icukncytosis ~ i t shift h to IrSt.. piier~tnr~iiia with I \ . Imaging I GTOSS Pathology Micro Pathology Treatment Discussion * HYPERSENSITIVITY PNEUMONITIS . P l T q : restriccivc lung disease pattern. called farmer's lung.~rninaterl Forced-air svrtrliil. aild cellt~ie~liarerl reartinns) .m p h o ~ i canrl s plnslnii c ~ l l s in nl\. B~-oncl~osct~pic I~iufi I>ir>psv r c ~ e i ~ interstilia1 ll.icei-bated when h e wnl-kl. G S R : bilateral retimlanodular inFi1trates with fihrnsis. serum antibodies against thermophilic A c t i n o rnp ups organisms: hronchonlrcr~lar l a n g r sllr~~cu ni:lr kctl I~rnpliocvt nqir. (t.. the most common Corm of hypersensitivity pneumonitis. Micro Pathology Treatment Discussion T h e rnain differential diagnoses to consicler are lung fibrosis aswciatetl with a cnilnective tissue disorder (rule nu1by history .A Ei5-yrar-c1ld ir~ale colnplai~ir nT pl-ojircssivc. l The onset of idiopathic pulinonat-v Fihmsis i s typically in the fifth or sixth rlecacle. Labs hl3Gs: hypoxernia.: clesaturatinn with exurcisc: p~-oporlional ely redt~cerl FEV. extrinsic alveolitis due to organic dusts. serum c. Imaging CSR: reticiilo~lodnlaishadow in both lower lung fields with ncc:isir~nalarea5 of "honeycombing. ant1 thickening of chc alvcolai. cough or-hrmoprysis. presence of 6hro3is. P!LT5: decreased DL.s. Rronchoscopically obtained lurig biopsy rwealc.sepba..PE: clubbing present. nnrmal calcium and ACE lcwls. inflan~matory round cell infilrrare. ncgative Kvei~n's test. a n d lack nC hilar 1yrnph. VS: rvann but cyanosed: tachycarrlia (HR 108)." CT (high I-esolt~tion) : lihrosis in Inwer lung 1ol)es suggestive of lislial interstitial pne~unonitis pattern of FPF. "The prjtient hns never smoked cigaretrr~ and has n o hirtory of exposure tn occupational dusts or fitnles.~nd clinical c-xam). tac!l)ipiiea: RP nor-ni. Atlas Link I D I O P A T H I C PULMONARY F I B R O S I S (IPF) . leftsided Elcart failure. l~rnnrhoalrenlar la~nge predominiinnrly neutrophilic. he has tint had a procluctivt.\l.respiratory examination reveals presence nf hilatcral basal fine inspiratory crepitations.rnul. shortness r l t ' I~reath on exer-tion and a chronic dry cough. a r ~ pneurnoccrnic>sis. ancl FVC so that ratio rc=rriairlecl~lnchangecl(due t o rertrictivc cli~ease). Jl'P not elevated: heart sounds ilormal with no a d c l i ~ i o ~sotincIs ~al or 1nltr.idcnopatliy ohrerved on CXR). lymphangitis carcinomatosa (rtite o u ~ on biopsy 2nd CT).~lci~inl ancl /ICE levels Eo~v. sarroidosis {rule nil1 otr the haris of ahscnce of anv othrr svsrern invofvei~~enl. q ttpex in ~ h superior c p~rlmr~nar .al syrtipa~hc~ic plex~ls.il'eil.has r x c ~ e r l e d brrilst carlrer a.t~lc~tr y tli. intercell~llar bridges.~.iqht 111 iilclle Inl>c.~) H PI H p has an 80-pack-year . Owins In rhe i~icrea~e incidellce d r)i srr~r>l.no breath sounds Ilea1d oyrr r-igh t middle Ir~he: vncal freiiiitlis rerlucrd in \ : 1 1 1 1 e .. Atlas Links r ' l PM-P2-085.tsivr m.mrl nlovemrnlq rliniiniuhed o n right.i the Iradi~rg c a u s r c~f cancer rteath irl women. dullness to percussion rtyel. anI~idrosis) :IT well as sci~pt~lar paill a ~ ulnar d ncrvc radiculc~pa~l-ty. Imaging CXR/C:T: i r r c ~ i 1 a 1 hililr nlass on tigfll side. anrl "keratin pearls.ccllit!:*rqtmtificatior~.tlut. normochrornic anemia. Pnnnu-gical sperimeil 1-eveaI4an irl-cpl.ics o f g~~"yi$li-tan tumor spreiidillg 0111 Fr-oril rig111 tniddle hronchns ai~d ul~~tructin il.4 . Ii~ng cancel.vic.lr inv. h Pancoast's trlmor i~a lilng nlnlor Ioci~tcd at tllr lu11. h hc has a l ~ o harl a significant loss of appetite and weight. T C PE 5 o z B 7 tabs CBC: normocytic.?Rye-ar-oEd trlalc prcsentr with shortness of breath hoarseness. resl~lting in Horner's syndrome (ptosis. Rroi1rhosct)pr: righl-sided liilar mass nhauucting right middle h r o n c l l ~ ~ s .irip. Marker1 pallor: cachexia. (rnl. niimis.~ I caltses corn presqintl nC the crr. clubbing: mild whet-zing at r r s t : c h r s ~ barrel shaped (ernphysrrnato~t~) . g Gross Pathology I Micro Pathology Diop~y revcaIs prcccncc o C n~alignanc squamaus rc511$. Clixrn a n d ZN r t x i n ~ rlf' spltturn fbr acid-f*ilstI~ilctll i neg:u ive: ylzlturn c l ~ c d rcveals o ~ presence of malignant squamow cells. pi~odiiring :III ohsunttiun iitrlec~asiu rlf r-ieht micldlp Inhe.smoking history. cough ancl hemoptysis.thc past 2 mor ~ t s." Surgici~l rcwc tion can hc p o ~ c n ~ i a Icurative lv in par ien ts wit 11 nun-smalIccll 111112 C C I I C ~ T .lD/CC . O v r r . PG-P2-085 ? LUNG CARCINOMA . :h. Treatment Discussion Lung cancrr is l h r most preventable cancer. A 67-yrar-nlrt tnak is referrecl to a clinic for evaluatint-i or pleuritic pain, weight 1 1 ~ 5pacl~ri~Fly , progre~sivedyspnea, and a nonproductive cough of a few rnon t h ~ rlz~ration. ' H e worker1 in ;I shipyard ror 20 years bcforc retiring. an orcupation that involved asbestos exposure. VS: normal. PS: clubbing of fmprs: mild c!lannsis; reduced chest expansion; end-i~~spiratory rnles auscultated over bath lung fields; c l d percussion, reduced breath sotmdq, and egophony in right side { ~ t lto ~ p1~1lml r errtlsion). C:RC/PRS: pnlycyt hernia; marked eosinophilia, PFTs: restrictive pattern observcd (dccrrased vital capacity and decreased total l t r r g capacity i\.ilIinr)rmal FEV,/F'VC; razio). Rcduced diffusion capacity; pleural efftl~ion bloody a11dshorvs acidic pH (< 7.3). Imaging CXK: rigllc-sided pleural effrisiou; diffuse bilaleml interstitial fibrosis; parietal pleural calcifications. T=T: highly i r i - u p l a r p lel~r-al-hafed rnasseq; 11 e n ~ o s s h a g i c effusion. Cross Pathology Thick, fibrous pleural plaques with calc=cation; diffi~se interstitial fibrosis: asbestos cc~rnpol~nd\ Form nesr for further c?epnuiiir,n nf il-or1 saltq and glvcoproteins ( m ~ l i r " r . r ~ c >ASRPSTOS us l3( ~IIIT:~). Micro Pathology Epi tlrelioicl pattrrn of pletu-al malignant sarcomatous t l - a n s f i l r m a t i o n with cellular atypia and high mitotic itldex. S l ~ s ~ ~poorr y : prognosis. Treatment Discussion Occlipationd exposure to asbestos is found in 80% :,C cases of malignant mesotheliorna; it produces lung fibrosis wit11 a : restrictive pattern. Ashcstos and tobdcco expnwr 1-e syn~rgisticallv i n c i - c a s c thc risk of lung ~ ~ L ' I I O C ; I ~ C ~ T ~ O I I ~ ~ . Atlas Links MALIGNANT MESOTHELIOMA ID/CC complaining OF pIeuritic pain o n the r i g h ~ side nf llcr clievt i111t1 dyspnea logether- will1 fever- ; n ~ d ; i prc~dllcti~ co~lgh. e , ; \ 57-yeal--old remale comes ro the ernergcnv room HPI TIi~re is n o hemnptvsis. T h e pail? i q nqicallv sharp and stabbing, a n d it nriws when 511e takes a deep Iwearh I K I ~ ~ ) . (r31.r:t L PE Decreased chest movement dtuing inhalation oil right side; dullness 011 percus5ion orright Iung base: reduced or absent breath sounds ores right lnng haw: I~ronchial I>rcath sounds in~sc~ tatctl t l 011 rig11t side; fr-iction ri~l>: Irrar ir )II r lf dullness moves with respiration; decreased tactile fremitus ovei- right f 0 z g < lung. Labs CBC: clevatrcl U'EC count with prerlorriinancr o i 'neutl-nphils. Gram-poriiive tliplr~coccinn qplltllrn smear and clzlt~lre: elevated protein, decreased glucose, and many neakophils in pleural exudate. Imaging CSR: c o n ~ l i d a t i o n of ri$t lower lobe: p1eur;il rff'i~sintl on right side. XI<. lateral ~ I e r ~ l ~ i layering i l ~ s : of fluid (tllereforc not loculated). Antibiotics ailcF ncecllc drainagu nf cffitsic~rl( T H O I L I ~ : I ~ ~ K ~ ~ - F . ~ I ~ } : tc~inrrtimer ol>litrl.a~ iot~ nf plrllral space. Plctrri~l cfft~sions rrlav be d u e lo iril'cc~ion(viral. har rerial, nlyrolmctc-rial, h11iq1):o t h r r ciillsrs are ttlal ig11:111cies, congestive heart fililw-e, cirrhosis, nrphrotic synclrvnlr. tmirna, p a ~ i c ~ - ~is, a tcollagen il tfi5e;irt.r. atlrl tf!-~lq reactioils. E1T11sions1 1 1 m Ile transudative (< 5 ~ / r l L aT PI-otein) or- exudative (> 3 g JdL of prolei 11). F,I~vatedpleural flilirl LDIH Irvcls mar. he s~~ggustive of n-lalignarrcv.Tms~adative pleural efrusions arc cornmonty citusrd hy conges~i\.e hyart failure, cil.rI~n\is, ant-1 nel~hrotic svndmmc, wherea~ exudative pleural effi~sions are c;titsed by TB, infections. malignancr: panel-ca~itis. pulmonary emlmll~s, a n d chvlothnrax (milky pleural fluid). Treatment Discussion PLEURAL EFFUSION u-hitc male complains or sudden pIe~tritic chest ID/CC h 2-i-yrar-r>Id pain and shortness of breath that awakens him at night. HPI I-Ie smokes onc pack of cigaretteq a clay and states that his patcrnaI uncle once had a similar episode. Tall, thin p a r i e n ~diapl~oretic ; and fuels wcali; lrft chest expands poorly o n inspiration; trachca and apex heat dirrplaced to right; left qide hyperresonant to percussiorl: decreawd breath sounds; demeaqed tactile fremitrrs. r\RC;s: rlecl-eased Po,; elevated Pc:o,. PE Labs Imaging CXR: partial collapse of left 111119 with no lung markings excupt thin line parallel to chest wan; costaphrenic sulcus a b n o m d y radiolucent ( ' ' I ~ E P P SI~L.CL~ SICS) S" in supine film. Types: rraumatic, sponmrieous, lension. open: common causes: s~irgical puncture, rLiplilrr of eniphvsematous bullae, positive pressure mechanical vet~tilatinn.hro~~choploural fistula. Pnrumothorax wacuatiori via p l e ~ ~ r a catherer l (r,~-rr:s~ TUBE). Gross Pathology Treatment Discussion T I he (rsual cause of spnn taneous pneumothoras is rupture of a subplerd ble b . air entPrs thc pleural spacc d ~ ~ r i i i g inupiratinn anrl i s pl-eve11 ~ r from d cqr-aping cl~rring coxpir-alio~l { l > e r s ~at1 ~.~n r\ltl.?-ossivt.ut r.~cliypnea. Flattrnecl lcft h c m i d i a p t ~ ~ + a ~ m .es) : right pneurnothorax compressing Ir~ng parenchyma and shifting of mediastinurn toward left. a~ thc c11c..fD/tC X -10-vrar-old III.c.. cheqi exam r ~ v c a l s l~yprrresonant percussion note on right.c i u lilletl with air anrl 1n1tgis aaelecta~ic( t o r l r r n o ~ ~ ~ t~ r~ i ~~ ~e eu l n c i t h o atl aurupqv.c..~chrcal-dia: t.. airwily ~ or ~~SFII flap C acts . PE: cyanosis. HPI Hc i\ . 1\113Gs: h!puxcmia: rc. S~ctir.: nnrmal. lettiilg ail huhl~les rrcape). t e i i ~ i o ~T l )e .cvurt=t.I ~ I I P C Idrain is n o r irnmcdiatelv availaMe. [ha[ m:m orcllr rluring prnitivr prcshure mechanicitl ~ e n ~ i l a t i n K ts ~k .paticrzl 41 ahil i. c y t i c Cil~rc lril.I c h ~ n t l i r smoker a n d has prerlolninal~tly emphysematous COPD.IS .IIP is Ijrn11g111 to [ h e ER nit11 cornplaintu c l f sudden-onset.Tife-saving trcxlment conqihrs o f 'illsrr~inga wiclv-horc o i l I l ~ ai7kriecl e sirlr I(> rlecor~~prcss thc pleural ci~tqtv if .l~yporrnsinn: n o few-r. severe right-sided chest pain followed by sevcre dirfic~llty breathing. F. 7 PNEUMOTHORAX-TENSION .. -D C PE I'S: . Immerlintc.a\rilv is npentd illnrlcr watcr. d t n i s h e d breath sounds. incrc-nw it1 [ ~ l e u ~air: x l whir11 is unrlcr pressul-c (i. rhr wiclr-hnr~ neetlle call then hc rep1:lcetl h~ a che\r drain cot~ilected to an nncln'watrr ~ r .inma. Plcnral 5piI. L:lr.rt)l-s for ~poiltxr~eotl? p r ~ c ~ ~ r n o t h o rincludc+ax COPD.piriltor 5 0 z 7~ > < Labs alkatosis.C:C.. .-I onc--wa~~ valve): rhel-ei s a pnq~. Imaging C X R (afttr.trachea shifted to left. l l .i Discussion 111 ~eilsionp~wrimntllorax. t ~ ~ pne11inot1lcrr-a~ i~n occllrs in onlv 1 tto 2% o r common manifestntiun of ilic barnts. GKOSS Pathology Micro Pathology Treatment I f ' c.inti decreased tactile fernih~s.noI I i ~ n a slinws collap~ecl a1vrc)lar spnccs. dizzirloss. parastcrnal heave. Atheromas in main elastic arteries.primary (irliopathic) or secondary to intrinsic pulmonary disease. ar causing near-oblit~ration uf the lumen. rLBGs: hvpoxemia. loud S2: narrow splitting of 52. ECC:: right-axis deviation. mles on both bases. Cabs Imaging CXR: enlarged light vcntriclc. ~ ~ l iqanosis.: rnlarged tnain pulmonary artery with peripheral pruning. and near-hinting spells. Primary plxlrnonary hypertension is a p a t h o l a ~ c incr-ease in pulmonary artery pressme. It may he. Gross Pathology Micro Pathology Treatment Calcil~m channel blockers: prostacyclin. if long-stanrling.ID/CC A 34year-old white obese female complains of shomess of breath. CBC: polycythemia. atherosclerosis or pulmonary artery. ld large "a" wave in jug-uIar venous pressure. inhaled raitric oxide. phlrhntomy: heart-lung transplaillation can he cnnsidered. HPI PE Obeqitv. i t raines latit1 right heart L~ilurc. Thickening of the media and intima in rnedilinl size m u s c ~ ~ larteries. narrc3wing of al-terioIes. Enlargccl right ventricle with mvocardial fiber hypcrtropky. right ventricle and right atrial hypertrophy. Shc has bee11 lltkirlg prescription medication for approximateIv 6 won ths in order to lose weight. hepatornega lv. Discussion Atlas Link U C D I PG-P2-090 P R I M A R Y PULMONARY HYPERTENSION . . CXR: right lo~+. Large thramhus scrn in pulmonary artrry. Pulmonary angiography is t i i t gold stanrl. Vircbow's triad ouilines 111c risk Lictors S i r thrombus forn~atior~ and inc-lucieq hlnocl Ftasis f r .'i: hypoxia and hypescapnia {type 2 respiratory rnilure). enrlor helid rlanlage ( e . Atlas Links * PULMONARY EMBOLISM . h ~ obtain ~ t a V/Q cran initially if clinically suspcctrd. 13:l o ~ ~ fever: ~ d . She has been immobile since the wr-gei-y. Imaging Gross Pathology Micro Pathology Treatment Discussion Pltlmnnal-y erlllboli mosr rommoiilv originate from pruxinlal deep venot 15 ~ h t . Sltpy>or-r ive. lid uudderr r l ~ . and low-molecular-~vcigl~t heparin ( cnclsapnrin ) insti tu terl for prop11ylaxi.c m hosis. LJS. Large ocrlz~sive I hl-omh ~ seen n in pi~lmonar-y artcrv wit11 mriabIc clegrcc of rrcani12ization. .n and sirills tachycardia.cllohc atrlcctasis. anrl hyperrnag-t~lahle states ( c .PE: r e n tral cyan osis: elevated JVP.t. FCC. ~ .i (monizc>rINR) . iriirnol1ili7alior1).g.. . Chun~adin. Doppler: clot in right common femoral vein.=C. Large emholi may c a u s c car~liovasculi~r cullapse I.~rdin tIlc rliawosis of pz~lrnntrary etnholism. ein hrhectoiny. ~lir-~!nboly tic 1hemp?: cot~sirler. Y J Q :thrue it1. surgery) . Labs .: SIQST3 pat1ei. ~ malignancy. heparin. hypotension. p l ~ l ~ n o r ~ a cor~fir. Anpjv.tachvcxr-dia: e tachypnea. prcgnarxcynsew-re I3~1rnsl.malol-y: ry: not reqnired il'Y/Q can is high probability.A ti(Lycar+lrl female who had ~ ~ n d c s g o n right e total hip replacement presents on t h e sixth pn5topcrativc day with ccnlral chest pain and acute-onset dyspnea.its of ventilation-prrft~sioi~ mismatch in right lung. ~ t h . right ventricular gallop rhythm with widely split 52. algia. Iiver. Imaging CXR: hilateraT hilar lymphadenopathy aucl rig11r paratracheal aclenopathv.~ vonos~mf): artIir. She has no tlistory of Careign traiwl or curltact with a. nocles. rvc. Corticosteroids. and erythernatouq.RI. HPI PE 15: Ccver. r pit lrelioirf cells. The diwast: rrlay ljc asyrnpturnatic: Iiowui-er. uppcr respiratory tr. and heart. including rhc l u n ~ slymph . syrnptoIrls rnay he cnnsti~~tintial a n d [nay iinvolvp many di Cf'eren t organ svstems. ilervow qstem. erythematous nodules over extenwr aspects of both legs (FR\TW. tZppl-oximatelp 60% to 7 1 3 % or sal-coidosispatients recover with T e ~ v or no residual wrnproms.a n s l ~ r c ~ ~1~ Lc I~ l~ IX bit ia~ l psv ordered.of hot11 knees: rpleno1nega1y Labs CBC: Iymphopenia. tender nodules on hnth legs. L. Mantoux test negative: 111ngal serolo~ negativc. arthralgia. PFTs: evidence of restrictive changes. dmnea.I D/CC A 28year4d black female cotnplai n F of fever. Treatment Discussion Atlas Link mPM-PZ-092 SARCOIDOSIS . eosinophilia. can llecnrne con fluent a n d gite lise lo larger nnrlulcl. hypercafciuria.v I?mpI tocyles.ytes: elevated serum calcium. 111 tlnc United States. ancl Lnt~gerlian's giant cells. skin. r~rticu1nendc)tlieliaI system.r.oticacell~rlar core surr-ounded l. with oTiset behveen 20 and 40 years of age. ACE levels elevated: 11Errnrl cultlrres negative. PF: tcn clcr.. Firm nt>clulcsonly a frw millimctel-sin size in affecrcd ol-ganq.acl. ~lzherc~~lar patient. Gross Pathology Micro Pathology Lbmp t~ node biopsy reveals noncaseatinig granulomas with fil>r. interstitial infiltrates: n o pleural eff~lsirsn. the incidence of sal-coidosis is highest in 13Tack wornen. kidneys. T ~ . ID/CC 115Tb1~ear=>TrE t~lalc.l ~ ~ n ill g s~ h early c T ~ ~ I ~FP~SI :P Xantl ~ hero~ne more rliff~ice as clistasi~ pr. Imaging Gross PathoZogy Del~ru. ~opaci~irc ll in tipper lobes with retraction anrl hilar Iyrnphadenapathy. his o c c ~ ~ l > a ~ i r11 .n istot-? .inetl ohsitrxrctive and restrictive pattern of functional iliip~irin~ I.sively LII increasing dyspnea xrlrl dry cough n o T severaI years' rlr~ralion. 7 C 0 Z f B m Labs PITS: cornl. or IympI~acterlc~patI~y reduced chest expansion 0x1 inqpirnrinn: d r y inspiratory crackles a~iscullated in upper Infwk of ht>th lrmgs. Irxrls to rrstriclivr l u l ~ g tlisease { h a t varie5 in wvriilv FI.c silica particles w i l l ~ i i tlo~lliles. + SILICOSIS . ~ l inrludes mining and quarrying.OTII ~iiilcl 10 dis:~l>li~jg. small c c ~ l l a ~ r i ~iznrlules c ~ l ~ u in the IIPPPI.~ldi?hes rlinfi-nnsi~: negative Mantoux i r s t : sputlun cvtr>lov:. Hc is a ~ ~ o r ~ s r n o kheu rt.. Hvalirlizetl whorl5 of c n l l a ~ e u ~ r . n Rrrjnch o~c~picallv-~uicIcr1 lung biopsv cst. 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