LOGOGangguan Eritrosit: Anemia dr. Desiana, SpPK., M.Ked (ClinPath) Gangguan Eritrosit LOGO Anemia Polisitemia ANEMIA LOGO Definisi Anemia: Sindroma klinis yang disebabkan penurunan massa eritrosit total dalam tubuh. Keadaan dimana massa eritrosit dan atau massa hemoglobin tidak dapat memenuhi fungsinya untuk menyediakan oksigen bagi jaringan tubuh Penurunan di bawah normal kadar Hb, hitung eritrosit, dan hematokrit ANEMIA LOGO Penurunan Hb dan Hct : < batas bawah 95% interval referens dari kelompok usia, jenis kelamin dan lokasi geografis (ketinggian) Hb12-14 g/dl ; (Hct 36-41%), Anemia Hb7g/dl symptom (+) Akut: hipovolumia (pucat, ggn penglihatan, syncope, tachycardia) ; Kronis: tissue hypoxia (fatique, dyspnea, Headache, angina) ANEMIA → symptoms / syndrome LOGO Hb ↓ PCV ↓ Hypoxia → Otak , Otot RBC ↓ Kompensasi : - heart rate ↑→ tachycardia → flow rate ↑ → cardiomegaly → heart failure → † - blood flow priority (pallor) - RBC 2,3-DPG content ↑→ O2 dissoc.curve shift to the right → O2 release to the tissues ↑ . 5 Klasifikasi Anemia LOGO Berdasarkan patofisiologi: I. Kegagalan produksi sel darah merah: A. Gangguan sel induk hematopoesis Anemia Aplastik B. Gangguan sintesis DNA Anemia Megaloblastik C. Gangguan sintesis Hemoglobin (Hb) Anemia Defisiensi Besi, Thalasemia D. Gangguan sintesis eritropoetin Anemia karena GGK Lanjutan…..anemia berdasarkan patofisiologi LOGO E. Gangguan karena mekanisme lain: Anemia karena penyakit kronis, anemia sideroblastik Anemia karena infiltrasi sumsum tulang II. Peningkatan destruksi sel darah merah: Anemia Hemolitik III. Kehilangan darah (Blood Loss) Anemia karena perdarahan akut Anemia LOGO Anemia berdasarkan morfologi Anemia sec. morfologi eritrosit, dilihat dari: - ukuran dan warna di bawah mikroskop atau - indeks eritrosit (MCV, MCH, dan MCHC) - Kriteria Ukuran (size): Normositik, Mikrositik, Makrositik - Kriteria Warna (pucat): Normokromik, Hipokromik Cara Mengetahui Ukuran eritrosit: LOGO * membandingkan dengan inti sel limfosit kecil (di bawah mikroskop) : → ukuran sama = normositik lebih kecil = mikrositik lebih besar = makrositik * Menghitung MCV (Mean Cell Volume) MCV= PCV/Ery X 10 (fL) (1 fL=10-12L= 1μm3) N : dewasa = 80-100 fL , di bawah 1 thn = 76- 86 fL MCV : normositik , mikrositik, makrositik * Eritrosit dengan variasi ukuran yang abnormal anisositosis 9 Bandingkan ukuran sel eritrosit dengan inti limfosit LOGO 10 LOGO 11 Perhatikan Warna sel eritrosit : LOGO - Bandingkan diameter central pallor(CP) dengan diameter sel eritrosit tersebut . - Normal, bentuk sel eritrosit adalah seperti cakram bikonkaf (biconcave disk) → pada hapusan darah tepi terlihat bulat, Ø 7-8 μ dengan area central pallor di bagian tengah CP≤ 1/3 Ø Eri = normokromik CP> ½ Ø Eri = hipokromik 12 Eritrosit dengan central palor (CP) LOGO Bandingkan diameter CP dengan diameter sel eritrosit 13 LOGO - Warna, dapat diketahui juga dari MCH (Mean Cell Hb) MCH= Hb/RBC x 10 (pg) Dewasa: MCH=27-32 pg, Anak-anak: MCH=23-31 pg (1pg=10-12g=1μμg) MCH normal → normokromik MCH < normal → hipokromik - MCHC (Mean Cell Hb Concentration) : MCHC=Hb/PCV x 100 (g/dL) Normal: MCHC = 32-36 g/dL 14 Klasifikasi Anemia secara morfologi LOGO 1. Anemia Hipokromik-Mikrositik. Anemia Normokromik- 2. Normositik Anemia Makrositik 3. LOGO Anemia Anemia Anemia makrositik hipokromik- normokromik- mikrositik normositik 1 2 3 Contoh: Contoh: A. Megaloblastik, - Anemia pasca contoh: - Anemia perdarahan akut - Anemia defisiensi defisiensi Fe - Anemia aplastik Folat, - Thalasemia - Anemia hemolitik - Anemia defisiensi - Anemia akibat - Anemia akibat vitamin B12 penyakit kronik B. Nonmegaloblastik Penyakit Kronik - Anemia pada GGK contoh: - Anemia - Anemia pada - Anemia pd peny. sideroblastik mielofibrosis Hati kronis - dll - Anemia pd hipotiroid, dll MCV <80 fl; MCV 80 -95 fl MCV > 95 fl MCH <27 pg MCH 27-34 pg www.themegallery.com Company Logo Hipokromik-Mikrositik LOGO 17 Normokronik-normositik LOGO 18 Makrositik LOGO makrosit-oval (Anemia megaloblastik ditandai oleh makrosit oval ini) 19 Pendekatan diagnostik Anemia: LOGO Anamnesis: onset /bleeding tendency / routine medicinal / occupation / hobby / travel history / family / diet / GI symptoms / menstruation cycle / history of previous pregnancy-delivery / alcohol consumption , etc Pemeriksaan fisik : conjunctiva & lips (pallor) / mouth (cheilosis) / tongue (glossitis) / gum / nails (koilonychia) , hair (signa de bandera, alopecia) , jaundice , petechiae , liver & spleen , lymphenodes ,rectal / vaginal toucher , feet (ulcer,arthritis) 20 LOGO Pemeriksaan Laboratorium - CBC (complete blood count )→ to confirm anemia (Hb, PCV, RBC) & the type of anemia (MCV; MCH; MCHC), RDW - Reticulocyte count → reflects marrow’s responses . - PBS : to look for the RBCs’ shape and any abnormalities of RBCs besides the other blood cell lines - Iron status ( Serum Iron ,TIBC, % Transferrin saturation , Iron storage ) - Blood chemistry ( direct/total bilirubin,LDH and stool examination for occult blood test , etc) . PBS: Pheripheral blood smear 21 Lanjutan…. Pendekatan Doagnostik… LOGO - Radiological examinations ( Chest X-ray, USG , MRI ) - Cardiological examinations (EKG,Treadmill, Echocardiography) Notes ! : - First confirm Anemia ( Hb , PCV , RBC ) - Classify the anemia (MCV, MCH, MCHC) - Causes of anemia 22 LOGO 23 LOGO 24 Anemia Hipokromik-Mikrositik LOGO - Setiap kondisi yang menimbulkan gangguan sintesis Hb gambaran hipokromik mikrositik - Anemia Defisiensi Besi penyebab tersering dari anemia Hipokromik-Mikrositik - Perhatikan penyebab lain (DD=diff diagnosis) sebelum mendiagnosis Anemia def. besi, spt: - anemia akibat penyakit kronis - Thalasemia - anemia Sideroblastik, dll 25 LOGO 26 ANEMIA DEFISIENSI BESI LOGO Definisi: Anemia yang timbul akibat kosongnya cadangan besi tubuh besi utk eritropoeisis pembentukan Hb Anemia def. Fe, ditandai dgn: - anemia hipokromik mikrositik - besi serum - TIBC (Total Iron Binding Capacity) - Saturasi transferin - Feritin serum - Pengecatan Besi sumsum tulang negatif - Respon terhadap pengobatan dengan preparat Fe LOGO Faktor Penyebab (Etiologi) LOGO I. Keseimbangan negatif Fe (Negative Iron balance): - Asupan Fe ↓ (inadequate diet , impaired absorption) - Fe loss ↑ (GI bleeding, excessive menstrual flow, bleeding diathesis) - ↑ demands (infancy, pregnancy, lactation) 29 Lanjutan….Faktor Penyebab LOGO II. Inadequate presentation to erythroid precursors: - atransferrinemia - Anti TrfR Ab III. Abnormal Fe balance : - Aceruloplasminemia - Autosomal dominant hemochromatosis ( mutations in ferroportin ) 30 Patogenesis desifisiensi Fe LOGO 3 pathogenetic factors: - Impaired Hb synthesis (consequence of reduced Fe supply) Transferin saturation< 16% inadequate Fe-supply to marrow → Hb contents of RBC ↓ → hypochromic & microcytosis - Generalized defect in cellular proliferation - Fe-deficient → oxidative damage to the red cell’s membrane → RBC deformability ↓ → RBC viability ↓→ RBC destruction ↑ especially in spleen → reduced RBC survival 31 Status besi tubuh: LOGO Serum Iron = SI Total Iron Binding Capacity (TIBC) % Transferrin Saturation = SI/TIBCx100% Simpanan besi (Iron storage): - Hemosiderin →produk degradasi feritin yang tidak larut dalam air → mayoritas tdd aggregat kristal ferric oxyhydroxide, FeOOH (di Hepar danSutul→ dideteksi dengan biopsi/aspirasi dan pengecatan besi (prosedur invasif) - Ferritin → kompleks garam Fe3+dan apoferitin yang larut dalam air, dengan jumlah yang sangat kecil di serum. (dideteksi dengan metode imunoasai) 32 LOGO Kandungan besi tubuh = 35-50 mg/kgBB: ±80% - Fe fungsional, sebagai heme-Iron (65% Hb, myoglobin, enzim heme : cytochrom-C,A,A3,B, catalase , peroxidase) - Non-heme-Fe (sebagian kecil) 20% - simpanan besi / Iron storage (ferritin, hemosiderin) hanya ± 15% pada wanita 0.2% - circulating (terikat padaTransferrin) 33 LOGO Iron Cycle in the body : Fe-diet → as heme-Fe (Hb, myoglobin, enzyme-Fe), 5-35% adsorbed from animal/meat sources , adsorbed easily . → as non-heme-Fe (vegetables , legumes), 90% of diet-Fe but only 2-20% of it absorbed → depends on the iron-status and the ratio of Enhancer:Inhibitor 34 LOGO Enhancers (zat yang menstimulasi penyerapan (absorbsi) : Ascorbate, Cytrate, organic acids / other amino acids , by reducing Fe3+ to Fe2+. Inhibitors (zat yang menghambat absorbsi) : Carbonate, Phytate, Tannins, Phosphate, Oxalat chelate Non-heme-Fe → unabsorbable 35 LOGO Bahan makanan yang menghambat absorbsi besi non heme (Non-heme Iron) : - Phytate (dari legumes, sayuran) - Tannin & Polyphenol (dari teh, kopi, wine, coklat ) - Phosphate/phosphoprotein dari kuning telur - Minerals (Ca, Zn, Cd) - Tetracycline yang bereaksi dengan Fe → menghambat absorbsi 36 Siklus Fe dalam tubuh : LOGO Diet’s Iron → duodenum / proximal jejunum . Iron from gut → released into circulation , bound to transferin → distributed to body’s organ / tissues( to bone marrow as a part of heme / Hb ) → circulate inside red blood cells with blood flow 37 The development of IDA LOGO • Stage-1 (prelatent Fe-deficient): - progressive loss of storage-Fe - body’s Fe reserve is still sufficient to maintain both the transport and functional compartment , so RBC development is still normal . - peripheral blood picture is normal , no symptoms of anemia , but ferritin is ↓ . *IDA= Iron Deficiency Anemia 38 LOGO * Stage-2 (latent Fe-deficient) - Exhaustion of storage-Fe , RBC production is still normal , Ferritin ↓↓ - Circulating-Fe (SI) begin ↓ , Transf- Receptor ↑ . * Stage-3 (Fe-Deficiency Anemia) - Stadium of Iron Deficiency Anemia 39 LOGO Stage-1 Stage-2 Stage-3 (prelatent) (latent) (IDA) Marrow ↓ (-) (-) Ferritin ↓ <12ug/L <12ug/L Transf-Sat N <16% <16% sTrfR N ↑ ↑ Retic Hb N ↓ ↓ content Hb N N < MCV N N < Symptoms fatigue fatigue pallor 40 LOGO Symptoms Morphology SI - TIBC Ferritin IDA Anemia Hypo – SI↓ - ↓↓ Micro TIBC ↑ A.C D Anemia Hypo – SI ↓ - N/ ↑ Micro TIBC ↓/N 41 Pendekatan Diagnostik Anemia Defisiensi Fe LOGO 1. Anamnesis – pola menstruasi, kehamilan / persalinan, tendensi perdarahan, penyakit kronis, diet, pekerjaan, riwayat bepergian 2. Pemeriksaan fisik – sistematik dari seluruh permukaan tubuh sampai ke organ dalam ( hati, limpa, kelenjar getah bening (lymphnodes) 42 LOGO 3. Laboratorium- Hema (DL, LED, Hapusan darah tepi, Retikulosit) - Serum (SI,TIBC,Ferritin, Bilirubin) - BMA (Bone Marrow Aspiration) - Pemeriksaan Urine dan tinja 4. Penunjang - Radiology (EKG, USG) - Endoscopy 43 LOGO SI TIBC Normal N N (1/3 mol.Trsf) IDA ↓ ↑ An.of Chronic ↓ N/↓ Disease Fe Overload ↑↑ N/↑ 44 Pemeriksaan Lab. Anemia def. Fe LOGO 1. CBC – confirm Anemia & find hypochromic microcytic picture from BSE and Red Cells Indices ( Hb, PCV ,MCV , MCH , MCHC) 2. SI – Fe2+ released from Transferrin + ferrozine (chromagen) → measured colored complex TIBC – serum + excess FeCl2 → to fill all Transferrin- binding sites → the excess Fe is fixed by Mg- carbonate → Fe-saturated Transferrin is measured with Ferrozine (= TIBC) 45 LOGO % Saturasi Transferrin = SI/TIBC X 100% Erythropoeisis impaired when % Tf.Sat < 15% 3. Ferritin Serum : Serum Ferritin level ~ Fe-storage Ferritin <15 ug/L → Definitive Fe-Deficient N/↑ Ferritin in IDA , if : - impaired liver function ( damaged hepatocyte), hemolysis, inflammation / infection / malignancy ( Ferritin = acute-phase protein ) 46 LOGO 4. Transferrin Serum : measured by immunodiffusion methode Normal value : 2-4 g/L 5. Bone Marrow’s Aspirate evaluation : ( using Perls or Prussian Blue stain ) 47 Anemia of Chronic Infection LOGO Gejala klinis miripdengan anemia def.Fe Gambaran lab. hematologi = Anemia def. Fe (An.Hypo-Micro, MCV↓, MCH↓, SI↓) , tapi TIBC N/↓ and Ferritin N/↑) Pathogenesis : Fe → storage // Transferrin Tissues / RES 48 Penyebab menurunnya ‘circulating Fe’ LOGO : 1. Impairment of Fe release from macrophage in competing with lactoferrin, phagocyte’s product , even storage-Fe is still enough . 2. Inadequate EPO Respons towards anemia (effects of cytokine production by macrophage) . 49 Diagnosis Anemia akibat penyakit kronis: LOGO lab hematologi: - Anemia hipokromik mikrositik - SI ↓ , TIBC ↓/N , Ferritin N/↑ ( jika Ferritin ↓, An. Def.Fe ) - Inflamasi / infeksi (+) : CRP and LED ↑ Problem: IDA with inflammation → ferritin ↑ (falsely diagnosed as ACD) ; it can be differentiated by sTfR exam (serum transferrin receptor) that ↑ in IDA but normal in ACD . 50 Anemia Sideroblastik LOGO Defek pada sintesis Heme → akumulasi Fe di mitochondria → degenerasi Fe → granula Fe di sekitar inti normoblast, membentuk struktur spt cincin {paling jelas terlihat dengan pengecatan Perl (Perls’ stain) } → Ringed Sideroblast (karakteristik anemia Sideroblastik) Sideroblast bisa dijumpai secara normal di sutul 51 LOGO Sideroblast and Ringed Sideroblast ( in Sideroblastic Anemia ) 52 LOGO 53 LOGO Classification of Sideroblastic Anemia 1. Hereditary : X-linked, defect in heme- synthesis enzyme pathway Fe absorption ↑ → % of Transferrin saturation and Ferritin level ↑ 54 LOGO 2. Acquired : - Primary : Stem cell clonal mutations(MDS = MyeloDysplastic Syndromes , RA-RS) Normochromic-macrocytic anemia . Marrow : erythroid hyperplasia with dysplastic or megaloblastic appearance - ringed sideroblast in normoblast . 55 LOGO - - Secondary; Abnormal metabolism of Vit.B6 (alcoholism, malabsorption) , impairment of heme synthesis ( Pb intoxication) , Rhematoid Arthritis , or An.megaloblastik . Usually related to myeloproliferative diseases ( AML, Myelofibrosis, Polycythemia or another types of MDS ) 56 Macrocytic Anemia LOGO - Non-Megaloblastic Macrocytic Anemia : Reticulocytosis Liver disease / Alcoholism Myelodysplastic Syndrome Erythroleukemia (FAB-M6) - Megaloblastic Macrocytic Anemia 57 Megaloblastic Macrocytic Anemia LOGO macrocyte = erythrocyte with MCV > normal . macrocyte/microcyte depend on the balance between nuclei & cytoplasmic maturation . (nuclear dividing stopped when intracellular Hb production reach a proper level ) . If nuclear maturation delayed ( in DNA synthesis’s defect ) or cytoplasmic maturation ↑ ( increase of EPO’s activities ) → critical level of Hb achieved earlier → Macrocyte 58 LOGO Megaloblast = bigger than normal normoblast . Megaloblastic changes = increased size of hemopoietic precursor cells in bone marrow ( not only in normoblast !) Primary defect : Defect of DNA synthesis ( altered almost all active cells / organs i.e : hemopoietic tissue, epithelial cells , mucous cells, etc ) 59 LOGO Etiology of DNA synthesis defect : deficiency of vit.B12 and folic acid → maturation dysharmony between nuclei & cytoplasm (delayed nuclei maturation) → increased cels (megaloblastic changes) → marrow’s ineffective erythropoiesis → intramedullary hemolysis → total/indirect Bili and LDH ↑. 60 LOGO Deficiency of Folic acid: - Inadequate diet (intake < / demand ↑ in pregnancy - lactation , child’s growth / malabsorption in tropical sprue / bowel resection / small intestine inflammation ) - Drug’s effect (anti-epilepsi) - FA loss ↑ (dialysis) 61 LOGO Deficiency of Folic acid: - Inadequate diet (intake < / demand ↑ in pregnancy - lactation , child’s growth / malabsorption in tropical sprue / bowel resection / small intestine inflammation ) - Drug’s effect (anti-epilepsi) - FA loss ↑ (dialysis) 62 LOGO Deficiency of Vit.B12: - Inadequate diet : Intake < in vegetarians , demand ↑ , impaired absorption caused by decreased Intrinsic Factor ( gastrectomy , pernicious anemia ) Malabsorption (bowel infection , worms / blind loop syndr ) 63 VITAMIN B12 ASAM FOLAT LOGO -Food from animal products -Limited sources (vegetable , -Heat stabile fruits) -Storage : enough for 3 yrs -Heat labile -Relatively low needs (only -Storage enough only for 3 1% of folate requirements) mths -Higher folate needs CAUSE OF DEFICIENCY CAUSE OF DEFICIENCY -Vegetarian (seldom) -Nutrition (alcoholism, goat’s -Impaired Intrinsic Factor milk diet) (pernicious anemia) -Prematurity -Gastrectomy -Hemodyalisis -Atropic Gastritis -Bowel resection -Anticonvulsant, alcoholism -Pregnancy -Anticonvulsant , MTX 64 Pathogenesis of Megaloblastic Anemia :LOGO Megaloblastic changes atrophy of tongue papilla & mucosal GI → glossitis , gastritis, nausea , constipation. B12 defic → demyelinisation of spinal cord & peripheral nerve → loss of foot’s balance / sensory (Neuropatia) FA defic → hyperhomocysteinemia → thrombosis and vascular occlusion . 65 B12 Metabolism LOGO Vit.B12 → purine & pyrimidin synthesis → synthesis DNA & RNA → mitosis and maturation Vit.B12 made from microbiological source because plants do not produce B12 ( meat , liver, eggs and milk are rich of Vit B12 ). Vit.B12 content in the daily diet is 5-3ug , daily requirement of B12 is 1-3 ug, and B12 body’s storage is 2-5 mg (enough for 3 yrs) 66 LOGO Vit.B12 absorption B12 diet → in gaster bind by IF (Intrinsic Factor) produced by parietal cells → IF-B12 complex → ileum : B12 absorbed , IF freed into the lumen impaired IF : gastrectomy/gastritis/ Auto-Ab-antiIF or Auto-Ab-antiparietal) → no absorption of B12 → impaired DNA synthesis → (Pernicious Anemia with Achlorhydria) Pernicious Anemia = autoimmune disease → auto- Ab to parietal cells (Anti-IF or Anti-Parietal) 67 Hematological pictures of Megaloblastic AnemiaLOGO Bone Marrow : - megaloblastosis - ineffective erythropoiesis Peripheral blood : - Oval macrocytosis - Hypersegmented neutrophil ( five 5-lobed cells or one 6-lobed cell) or the mean lobes of 100 neutrophils is > 3.4 68 Megaloblastic Anemia LOGO find oval-Macrocyte cell and hypersegmenteneutrophil . 69 Diagnosis of Megaloblastic Anemia LOGO Screening : - CBC , Neutrophil’s lobe count - Serum Indirect Bilirubin , LDH (lactate dehydrogenase) Spesific tests : - Bone Marrow Aspiration: megaloblastosis & megaloblastic changes, erythropoietic activitiy ↑ ( ineffective erythropoiesis) - Folate & Vit.B12 assay - Gastric juice analysis - Schilling Tests - Antibody Assay 70 Anemia Hemolitik LOGO Anemia hemolitik: anemia yang disebabkan oleh proses hemolitik. Hemolisis: pemecahan eritrosit sebelum waktunya (sebelum masa hidup rerata eritrosit, yaitu 120 hari). (Proses pemecahan eri karena sdh waktunya senescence=penuaan) Hemolisis dapat terjadi di dalam pembuluh darah (hemolisis intravaskular) dan di luar pembuluh darah (hemolisis ekstravaskular). www.themegallery.com Company Logo HEMOLYTIC ANEMIA LOGO Normal red cell’s survival = 110-120 days → destructed by macrophage in marrow and spleen . When the survival are shortened → EPO production is stimulated (compensated) → no Hb changes → anemia (–) . If the destruction is acute or chronic with very shortened life of red cells , there will no compensation → anemia (+) . 72 Definition of Hemolytic Anemia : LOGO anemia caused by shortened red cell’s survival as a result of excessive uncompensated destruction of red cells . Hemolytic process = every process of red cells destruction with still / without compensated by bone marrow → anemia is not always present . 73 - Compensation ability of bone marrow LOGO : Ability to ↑ red cells production ( 6-8 x normal ) : - survival shorten ½ → production ↑ 2x - survival shorten ¼ → production ↑ 4x - survival shorten 1/6 → production ↑ 6x - survival shorten 1/8 → production ↑ 8x ↑ of production 6-8 x is maksimum . If red cells live only 20 days → anemia (+). 74 Diagnostic approach in Hemolytic Anemia LOGO : 1. Confirm anemia (Hb/PCV/RBC) an acute case usually acquired , and chronic case is mostly hereditary . 2. To find the signs of hemolytic process . 3. Extra or Intravascular ? 4. Hereditary or acquired ? 5. The cause of hemolysis episodes . 75 The signs of Hemolytic process : LOGO 1. Increased of red cells destruction - Unconjug.bilirubin serum ↑ → jaundice - Urobilinogenuria - Hb-uria → sign of intravascular hemolysis - Abdom.pain → splenomegaly, spleen infarction - Leg’s Ulcer → intrinsic defect of erythrocyte - Haptoglobin serum ↓↓/neg → intravascular hemolisys . 76 LOGO 2.Destruksi eritrosit : - Microspherocyte, Fragmentocyte, Poikilocyte - Erythrocyte Osmotic Fragility ↑ - Positive Autohemolysis test - Shortened of red cells’ survival 3. Tanda Peningkatan Eritropoisis: - Reticulocytosis - Normoblastosis - Erythropoietic Hyperplasia in bone marrow 77 LOGO 78 LOGO 79 LOGO 80 LOGO 81 Hemolisis Ekstra vaskular LOGO Hemolisis ekstravaskular lebih sering dijumpai dibandingkan hemolisis intravaskular Hemolisis terjadi di sel makrofag dari sistem retikuloendothelial (RES) terutama pada Lien, hepar dan sutul karena sel ini mengandung enzim heme oksigenase Lisis terjadi karena kerusakan membran eritrosit (misal Akibat reaksi Ag-Ab; presipitasi hb di sitoplasma, menurunnya fleksibilitas eri,dll) LOGO 83 LOGO 84 LOGO 85 Klasifikasi Anemia Hemolitik LOGO Dibagi atas 2 golongan besar, yaitu: 1. Anemia hemolitik karena faktor di dalam eritrosit sendiri (gangguan intra korpuskuler) 2. Anemia hemolitik karena faktor di luar eritrosit (gangguan ekstra korpuskular) www.themegallery.com Company Logo lanjutan….Klasifikasi anemia hemolitik : LOGO 1. Gangguan intra korpuskular (Hereditary Hemolytic Anemia ) - Membrane abnormality (hereditary spherocytosis , hereditary ovalocytosis ) - defect of globin chain (Thalassemia, Hb- pathia) - enzyme defect ( G-6PD deficiency , PK- deficiency) 87 Hereditary Spherocytosis : LOGO 88 Hereditary Ovalocytosis : LOGO 89 Lanjutan……klasifikasi anemia hemolitik LOGO 2. Gangguan ekstrakorpuskular (Acquired Hemolytic Anemia): - physical / chemical substances - infections (bacteria, parasites, viruses, fungi) - mechanical trauma (prostetic heart valves) - Immune mechanism (Alloimmune / Autoimmune / Drug-Induced HA) 90 - Hereditary Spherocytosis : LOGO autosomal dominant Spherocytosis, decreased membrane surface area relative to cell volume → osmotic fragility test (OFT)↑ among the family member . The primary lesion is caused by membrane protein defects (↓of spectrin) → cytoskeleton instability . 60% - chronic anemia , jaundice, splenomegaly, 20% without hemolysis / splenomegaly . Bilirubin excretion ↑ ,causing bilestone in USG. 91 Thalassemia : LOGO Defect of 1 or more globin-chain synthesis (the amount = quantitatively) : - deficiency of α globin-chain → α-thalassemia - deficiency of β globin-chain → β-thalassemia - deficiency of δβ globin-chain → δβ-thalassemia the primary defects in Hb-pathia is in the globin amino acids structure (qualitatively) 92 LOGO 93 LOGO 94 α-Thalassemia LOGO α-Thalassemia = is caused by the impairment of α-globin chain production/synthesis . α-globin chain synthesis is directed by 2 pairs of α-gene (4 locus α-gen) → depending of the number of defected locus → 3 types of α-Thalassemia (α-thal trait , HbH Disease, and HbBart’s Hydrops Fetalis) 95 Clinical consequences in α-Thalassemia LOGO Deficiency of α-globin chain → excess of β, γ chain since fetal life to form β4-tetramers (HbH) or γ4-tetramers (HbBart) . Defect of 1-2 α-Gen = α-trait (clinically good) Defect of 3 α-Gen = HbH disease ( Hb 10-11 g/dl) → excess of β-chain → to form β4- tetramers (HbH) as intracellular inclusion → detected by BCB-stain . 96 HbH-inclusion (β4) in HbH Disease as shown in BCB staining (compare with reticulocyte)LOGO 97 LOGO Defect of 4 α-gene (HbBarts’hydrops fetalis) → clinically severe , stillborn baby with hydrops fetalis ( severe hypoxia ) . HbBarts = γ4-tetramers (excess of γ-chains that unable to form HbF ) . HbBarts and HbH inclusions precipitated in red cell’s membrane → mechanical trapping in spleen → macrophagic phagocytosis → hemolysis . 98 LOGO 99 LOGO 100 - β-Thalassemia LOGO Clinically consequences in β-Thalassemia : - No problems during fetal life because HbF synthesis is normally produced (normal α and γ chains) - When HbA is dominantly needed , the clinically problems exist as incapability to synthesize HbA (α2β2) → excess of α-chain → compensated ↑ of δ and γ production → HbA2 ↑ (in β-Thalassemia minor) and HbF ↑ (in β-Thalassemia mayor) 101 Β-Thalassemia mayor : LOGO - severe anemia → repeated transfusion is oftenly needed → Fe↑↑ → hemochromatosis - chronic ineffective erythropoiesis → medullary hypertrophy in childhood → facial malformation: * Frontal bossing * Maxillary hypertrophy * Hypertelorism (mongoloid’s eye) 102 - β-chain deletion forms : LOGO β0-Thalassemia : no β-chain production. β+Thalassemia : β-chain production << in heterozygous case : medium severe in homozygous : severe (Cooley’s anemia) 103 LOGO 104 Laboratory Diagnosis in Thalassemia LOGO 1. CBC, Peripheral Blood Smear 2. Hb-Electrophoresis : in Celulose-Acetat (pH 8.4) for thalassemia and Hb-pathia screening Using hemolysate → formed bands of different types of Hb ( normal : bands A, F, and A2 , measured densitometrically) 105 LOGO 106 Lanjutan…..Lab diagnosis in thalasemia LOGO 3. HbA2 mesurement to diagnose β-Thalassemia trait using anion-exchange resin column chromatography in both HbELP and chromatography , HbC, HbE and HbO can interrupt the conclusion because of the same band location with HbA2 . 4. HbF determination : - Alkali Denaturation Test - Acid-elution (Kleihauer) test - RID or ELISA methods 107 LOGO 5. HbH Inclusion detection : - Supravital staining using Brilliant Cresyl Blue (BCB) or NewMethylene Blue (NMB) - HbH inclusion seen as dispersed blue- green granules in red cells (compare with reticulocyte as a filament) - in HbH disease : HbH inclusion +++ - in Thalassemia-α-trait : HbH inclusion + in 1: 10000 eritrosit . 108 Defisiensi G-6PD LOGO - Oxidant → produce H2O2 → oxidizing Hb’s free sulfhydryl → to form Sulf-Hb → aggregates that precipitated as Heinz Bodies → destructed in spleen . - Oxidant / Sulf-Hb are controlled by Reduced Glutathione (GSH) 109 LOGO 110 LOGO - X-linked, ± 300 variants . normal G-6PD genes : - type B (GdB) - type A (GdA) - Abnormal enzyme types : 1. GdA– (type A–) 2. Gd-Mediterranean (GdMed) 3. Gd-Canton : many in Asia - G-6PD deficient red cells are resistent to Plasmodium Falciparum . 111 LOGO - Substances causing lysis in G-6PD deficiency : 1. Antimalaria 6. Fava beans 2. Sulfonamides 7. Naphtalene 3. Vit.K, Vit.C 8. Uremia 4. Lung Infection 9. Antibiotics (virus,bacteria) (Penicilline , 5. Antipyreticum streptomycine 112 LOGO The highest G-6PD activity is in reticulocyte . G-6PD screening test : Test’s principle : G-6PD G-6P + NADP 6-PG + NADPH UV (fluorescence) 113 LOGO Acquired Hemolytic Anemia : - Secondary Hemolytic Anemia caused by infection / systemic disorders : Malignancy – Autoimmune-reacted hemolysis , microangiopathy or hypersplenisme , appearing Anemia of chronic disease, bleeding tendencies, and marrow’s suppression 114 LOGO Disseminated Intravascular Coagulation (DIC): Systemic intravascular coagulation → fibrin deposit intravascularly / endothelial damage (microangiopathyi) caused by sepsis → red cells destruction . Chronic Liver Disease : hemolysis caused by hypersplenism . Chronic Renal Disease: hemolysis caused by microangiopathy 115 Acquired Hemolytic Anemia (extracorpusc.) LOGO Immune Hemolytic Anemia Red cell membrane-bound Ab hemolysis . The speed & hemolysis location depend on IgG or IgM, and the ability to activate complement . Optimal temperature to bind Ab : 370C – Warm-IgG-Type <300C – Cold-IgG-Type 116 Lanjutan….acquired hemolytic anemia LOGO Cell+IgG → destructed by spleen Cell+IgM → enhance the activation of complement’s cascade → intravascular hemolysis Immune destruction often cause minimally membrane damage → shape change into spherocyte . 117 LOGO Immune Hemolytic Anemia classification : 1. Alloimmune : Transfusion Rx , Hemolytic Disease of the Newborn (HDN) 2. Autoimmune : Warm/Cold AIHA, Paroxysmal Cold Hb-uria (PCH) 3. Drug-induced HA : penicilline type, aldomet, and stibophen type . 118 Hemolytic Disease of the Newborn (HDN) – Rh-neg mother , with Rh-Pos fetus , during I and second LOGO pregnancy 119 Antiglobulin Tests (Coombs) : LOGO Direct Coombs Test (Direct Antiglobulin Test/DAT) = Ab detection test (IgG and or C3d /complement-bound red cells) . Indirect Coombs Test = test for serum free Ab . DAT usually positive in AIHA (. 120 Drug-Induced hemolytic anemia : LOGO Penicilline type : drug as hapten binds red cell membrane → antigenic → stimulate Ab production against Drug in drug-red cell complex Phenacetin/Quinidin type : Drug (hapten) adsorbed protein → stimulated-Ab binds drug-protein complex → activate complement → red cell lysis. Aldomet type : drug change red cell membrane’s structure → detected as foreign cell → Autoantibody production . 121 LOGO 122 Aplastic (Hypoplastic?) Anemia LOGO Severe & fatal Anemia because of ↓ red cells/leucocytes/platelet production (pancytopenia) caused by Stem Cells impairment (radiation, chemicals, drugs, or genetic matters) Marrow aplasia / hypoplasia-causing substances - radiation , benzene, cytostatics (6-MP, busulfan), arsen, chloramphenicol, anticonvulsant (phenytoin), analgetic (phenylbutazone) , DDT, etc 123 LOGO Symptoms & Lab.appearance of Aplastic Anemia fatigue, palpitation, infections, bleeding tendency Lab : - pancytopenia - normochromic normocytic - ‘dry-tap’ marrow , hypocellularity Prognosis : - bad especially for < 40 yrs old patients → marrow transplantation . 124 - Treatment for Aplastic Anemia : LOGO 1. Avoid every toxic material 2. Avoid infections / bleeding tendency 3. Use Washed-Erythrocyte if transfusion is needed or Plat.Concentrate (PC) for any profuse bleeding ( give corticosteroid if bleeding is minimal) 4. Marrow stimulants (androgenic hormon ) 5. Marrow Transplantation 125 POLISITEMIA LOGO (ERITROSITOSIS) Peningkatan patologis massa eritrosit massa eritrosit normal : (sea level) - o : 26 - 32 ml / kg BB - o : 23 - 29 ml / kg BB eritrositosis : massa eritrosit > normal ( PCV : o >51% ; o >48% ) LOGO • Klasifikasi : I. Primer (Otonomik) A. Polisitemia Vera B. Eritrositosis Murni (Eritremia) II. Sekunder A. Fisiologis (Oksigenasi Jaringan ) B. Non-fisiologis (Oksigenasi Jaringan N) III. Eritrositosis Relatif ERYTHROCYTOSIS - DIAGNOSTIC TESTS LOGO • Complete Blood Count • Bone Marrow examination • Arterial Blood Gas analysis • Leukocyte Alkaline Phosphatase • P5O • IVP or renal ultrasound • Liver ultrasound or CT scan • Erythropoietin level • Erythroid progenitor assay • Sleep apnea evaluation LOGO POLISITEMIA VERA • Proliferasi klonal neoplastik sel progenitor hematopoitik pluripoten • Kriteria diagnosis P.V. : Kategori A 1.Massa eritrosit: Lk > 36 ml / kgBB (PCV > 54%) Pr > 32 ml / kg BB (PCV > 51%) 2. Saturasi oksigen > 92% 3. Splenomegali LOGO Kategori B 1. Trombositosis (> 400.000 / ml) 2. Lekositosis (> 12.000 / ml) 3. Skor LAP 4. B12 serum > 900 pg/ml • Diagnosis PV + bila : +A2 ++ A3 + atau A1 + +A2 ++ dan 2 dari kategori B A1 + + LOGO PRIMARY “PURE” ERYTHROCYTOSIS ( ERYTHREMIA ) • peningkatan massa eritrosit murni • tidak ada penyebab eritrositosis sekunder • kadar eritropoitin normal atau rendah • mungkin akibat mutasi gene reseptor eritropoitin progenitor eritroid jadi lebih sensitif terhadap eritropoitin. LOGO II. ERITROSITOSIS SEKUNDER • Merupakan respons terhadap keadaan lain yang bersifat : - fisiologis : akibat oksigenasi jaringan yang - non fisiologis : tanpa penurunan oksigenasi jaringan LOGO III. ERITROSITOSIS RELATIF • Sindroma Gaisbock • Stress erythrocytosis • Pseudo erythrocytosis - Massa eritrosit tinggi normal - Volume plasma rendah LOGO www.themegallery.com Click to edit company slogan . SOAL LATIHAN : LOGO 1. Nyonya Ana, usia 40 tahun, MRS (Masuk Rumah Sakit) dengan keluhan pusing, dan badan terasa lemah. Pemeriksaan fisik: KU lemah, Tensi: 100/60 mmHg, Nadi:90 x/menit, RR: 20 x/menit, suhu:37˚C. Kepala/Leher: anemia (+), tidak dijumpai ikterus, dyspnea dan sianosis, Thorak/Cor dan Abdomen :dalam batas normal (dbn). Extremitas: dbn. Hasil laboratorium: Hb 8 g/dl, RBC 3,20 x 1012/L, Hematokrit 24 %, MCV 75 fl, MCH 25 pg, MCHC 33 g/dl. Jika anda adalah dokter jaga di RS tersebut, dari data yang ada, kemungkinan diagnosis pasien tersebut adalah: A. Anemia normokromik-normositik B. Anemia hipokromik-mikrositik C. Anemia makrositik D. Anemia makrositik-megaloblastik E. Anemia makrositik-non megaloblastik www.themegallery.com Company Logo Lanjutan …...soal latihan LOGO 2. Dari kasus ny. Ana, 40 tahun tersebut, diagnosis diferensial untuk penyebab anemianya adalah: A. Anemia defisiensi folat, anemia defisiensi Vitamin B12, B. Anemia karena perdarahan akut, anemia aplastik C. Anemia defisiensi besi, thalasemia, anemia sideroblastik D. Anemia hemolitik, anemia pada penyakit mielofibrosis E. Anemia pada penyakit liver, anemia pada penyakit hipotiroid www.themegallery.com Company Logo Lanjutan …...soal latihan LOGO 3. Dari soal kasus Ny. Ana, 40 tahun tersebut, langkah pemeriksaan laboratorium selanjutnya yang perlu dilakukan untuk konfirmasi diagnosis adalah: A. pemeriksaan bilirubin, haptoglobin, hitung retikulosit B. Serum Iron, TIBC dan Feritin C. Pemeriksaan B12 dan asam folat dalam darah D. Pemeriksaan T3, T4 dan TSH E. Pemeriksaan Aspirasi sumsum tulang www.themegallery.com Company Logo CLINICAL CASE LOGO A 35-year-old man complains of chronic physical fatigue, which began 3-4 weeks ago. He said he felt tired all of the time even through his occupation as a software developer was mentally but not physically demanding. He breathed comfortably at rest but, when he exerted himself, he experienced difficulty in breathing and had hard time catching his breath. He also complained of „more than usual” mental fatigue, confessing an increasing inability to concentrate and focus his attention on tasks at hands. Colleagues noticed his pallor and his inattentiveness at brainstorming sessions and suggested he reschedule his annual physical examination for an earlier date. He complained of vague abdominal pain and sense of abdominal fullness. His appetite was depressed, and he thought perhaps his physical and mental symptoms were caused by poor diet. However, attempts to increase eating resulted in nausea. His stools, he said, were sometimes loose and tarry. Eventually, increased heart palpitations and chest pain made him seek medical advice Laboratory findings revealed the LOGO following: Laboratory test Patient Normal RBC (red blood cell count) 3.5 T/L 4.5-6.0 T/L HCT (hematocrit ratio) 28% 40-52% Hb (hemoglobin) 8.0g/dL 13-17g/dL MCV (mean corpuscular 70fL 78-95fL volume) MCH (mean corpuscular 22.8pg 29pg hemoglobin) MCHC (mean corpuscular 28% 34% hemoglobin concentration) QUESTIONS LOGO Case history questions: 1. What general medical condition is suggested by the person’s symptoms? 2. What fundamental change in function of blood related to the red blood cells could simultaneously affect the function of several systems (cardiovascular, respiratory, gastrointestinal, and others)? 3. What specific diagnosis is supported by the laboratory findings? 4. How could the stool be related to the laboratory findings? ANSWER LOGO Answers: 1. Anemia 2. A reduction in oxygen-carrying capacity of the blood and thus a reduction in the delivery of oxygen to various body tissues 3. An iron defficiency anemia 4. Most cases of iron-defficiency anemia result from internal blood loss. Dark, tarry loose stools suggest bleeding from the gastrointestinal tract and warrant further tests to determine the exact cause LOGO TRANFUSI DARAH www.themegallery.com Company Logo KOMPONEN DARAH LOGO Darah Lengkap ( WB ) * Perdarahan akut ( > 20 %) Hb < 10 Gr %, PCV < 30 % * Total blood exchange ( neonatus ) Pack Red Cell * Anemia kronik * Pre operative Hb < 10 gr % Washed Red Cell * Alergi terhadap protein plasma * Hemodialisis Deep Freezing Red Cell * Alergi terhadap plasma, lekosit, trombosit www.themegallery.com Company Logo LOGO Lekosit • Netrofil < 200 / mm3 dengan bukti infeksi bakteri / jamur yang tak terkendali dengan AB selama 48 – 72 jam Trombosit * Trombositopenia dengan perdarahan : < 100000/ mm3 * Trombositopenia : < 40000 / mm3 * Preoperative dengan trombosit : < 100000 / mm3 Plasma * Defisiensi fc II, V, VII, IX, X, XII, XIII * Perdarahan akibat antikoagulan warfarin * Transfusi darah masif yang disertai koagulopati www.themegallery.com Company Logo DONOR DARAH LOGO 1. SEHAT DOKTER 2. SUKARELA 3. USIA 18 – 65 TAHUN 4. FREKWENSI 2- 3 KALI SETAHUN 5. VOLUME MAKS. 13 % www.themegallery.com Company Logo Cross - Matching LOGO * ABO O A B AB Antigen (-) A B A+B Eritrosit Antibodi Anti-AB Anti-B Anti-A (-) Serum •Rh + / - Screening antibodi www.themegallery.com Company Logo Uji Saring Untuk Pendonoran Darah LOGO Mutlak di Inggris • Antigen permukaan hepatitis B • Antibodi terhadap HIV-1 dan HIV-2 • Antibodi terhadap Treponemapallidium (sifilis) • Antibodi terhadap virus hepatitis C Relatif (resipien tertentu) : • Antibodi terhadap sitomegalovirus Indonesia • HVB, HVC, HIV, VDRL www.themegallery.com Company Logo REAKSI TRANSFUSI LOGO Definisi : Komplikasi / efek samping yang terjadi akibat pemberian transfusi Klasifikasi Imunologi : Produksi anti bodi terhadap aloantigen pada eritrosit, leukosit, trombosit atau protein plasma darah Non Imunologik : Berhubungan dengan bahan fisika/kimia komponen darah atau kontaminan www.themegallery.com Company Logo Klasifikasi Lain LOGO Rx transfusi cepat - Rx demam - Rx alergi urtikaria, anafilaktik - Rx hemolitik cepat - Bakterimia / septik Rx transfusi lambat ( > 48 jam ) - Rx hemolitik lambat, purpura post transfusi, GVHD Circulatory overload Penularan penyakit www.themegallery.com Company Logo Reaksi Imunologik Pada Transfusi LOGO Darah Komponen Penyakit Persentase Eritrosit Rx Hemolitik -Segera 0.02 % -Lambat 0.2 % Leukosit Rx Demam 5 – 10 % Edema Paru akut non kardiogenik < 0.01 % Trombosit Purpura pasca transfusi < 0.01 % Protein plasma Natif Anafilaktik < 0.01 % Tertelan Urtikaria 1.3 % www.themegallery.com Company Logo I. Reaksi Terhadap Eritrosit yang Tak LOGO Cocok Eritrosit Darah + Antibodi Resipien Aktivasi Komponen C3a KID Hemolisis C5a Deposit Deplesi Vasodilatasi Fibrin Hemoglobin Faktor koagulasi dan Trombosit Hipotensi Hemoglobinuria Gagal Ginjal Perdarahan www.themegallery.com Company Logo LOGO Rx Segera ( Hemolisis intravascular ) Penyebab Inkompatibilitas ABO ( 86% ) Tu gol. O Kontaminasi darah donor Suhu penyimpanan terlalu rendah / tinggi Tahapan Fase Syok Hemolitik Demam, menggigil, nyeri kepala/punggung/dada/ekstremitas/ flushing, sesak, mual muntah, takikardi, syok. Fase Pasca Syok ( 12 jam ) Anemia, leukositosis, ikterik Fase Oliguri ( 6 – 12 hari ) Fase Diuretik ( Beberapa hari ) Rx Lambat ( Hemolisis LOGO Extravascular ) • Antibodi eritrosit yang tak terdeteksi anti JK, anti Rh, Anti K, Anti Fy • Rx imun sekunder • Eritrosit yang diselimuti IgG akan dimakan makrofag • Demam, ikterus, hemoglobinuria Penatalaksanaan Rx LOGO Hemolitik Segera Hentikan transfusi Pertahankan status hidrasi Pertahankan produksi urine 100 ml/jam, dapat diberikan furosemid 80 – 120 mg IV Obat vasoaktif : dopamin Bila didapatkan koagulopati : heparin, transfusi komponen (FFP, Kriopresipitat, trombosit) Terapi gagal ginjal ; restriksi cairan, keseimbangan elektrolit, dialisis Penatalaksanaan Rx LOGO Hemolitik Lambat Tidak ada terapi spesifik Pada reaksi berat terapi sama dg Rx hemolitik segera Evaluasi : - hemolisis : bilirubin,heptoglobin - aloantibodi - KID fungsi ginjal - pseudohemolytic transfusion reaction II. Rx Terhadap Leukosit yang Tak LOGO Cocok 1. Rx Demam ( 56 % dari RX transfusi ) Leukosit Asing + Antibodi Leukosit Ditelan Monosit Resipien Pembebasan Pirogen Demam Juga terhadap trombosit, plasma Thy/ = Stop Transfusi = Antipiretik, Kortikosteroid = Evaluasi Hemolisis, Kontaminasi Bakteri/toksin www.themegallery.com Company Logo 2. Edema Paru non Kardiogenik LOGO Antibodi Plasma Donor VS – HLA, antigen granulosit Spesifik Aglutinasi Granulosit Aktivasi Komplemen Kerusakan Endotel Kapiler Paru Transudasi Cairan di Alveoli Thy/ - Suportif - Monitor Hemodinamik - Steroid Dosis Tinggi - Evaluasi Plasma Darah www.themegallery.com Company Logo III. Rx Terhadap Trombosit yang Tak Cocok LOGO Purpura Pasca Transfusi Rx aloantibodi terhadap antigen trombosit ( HPA – 1 = Human platelet antigen ) 2 – 10 hari pasca transfusi TERAPI Ig G dosis tinggi dan atau plasma exchange Kortikosteroid www.themegallery.com Company Logo IV. Reaksi Terhadap Plasma LOGO Antigen Protein Plasma VS Ig E Resipien Urtikaria Antigen Protein Plasma Vs Ig A Resipien Reaksi Anafilaktik Thy/ Ringan – Transfusi dilambatkan, antihisatamin Berat – Transfusi dihentikan, Thy Rx anafilaktik www.themegallery.com Company Logo 1. Bakteridan Parasit yang Dapat Ditularkan Melalui Transfusi Darah LOGO Bakteri pseudomonas,salmonella Bruselosis Spilis Parasit Plasmodium (malaria) Trypanosoma cruzi (penyakit cagas) Endemik di Amerika Latin. Toxoplasma gondii Babesia microti (demam nantucket) Resiko potensial di Amerika Utara Virus HVB, HVC, HIV, HTLV-1, CMV, EBV Rx Bakteremia / Sepsis LOGO Darah tercemar bakteri E. Coli, Proteus, P Aeruginosa, K Pneumonia Gejala sudah timbul saat darah masuk 50 cc Demam tinggi, menggigil TD menurun, syok Mual, muntah, nyeri seluruh tubuh DIC Dx pasti : Kultur darah sisa Penatalaksanaan Stop transfusi, selainnya sesuai syok sepsis www.themegallery.com Company Logo 2. Volume Overload LOGO Gagal jantung akut Risiko tinggi - Anemia kronis - orang tua - kelainan jantung/paru/ginjal Pencegahan - PRC / 2 jam - Pre Furosemid 1 ampul Thy - Sesuai gagal jantung akut 3. Transfusi Masif LOGO * Pemberian lebih dari 1 volume darah dalam waktu < 24 jam * Perdarahan hebat kecelakaan, operasi, obgyn - Hiperkalemia, hipokalsemi, hipotermi - Trombositopeni, koagulopati Thy/ Substitusi (belum ada kesepakatan) 4. Hemosiderosis LOGO 1 unit darah → 0.2 gr Fe Menumpuk di Jaringan Gangguan pertumbuhan Disfungsi miokard / hepar Diabetes Hiperpigmentasi Thy/ Iron chelating: desferoxamin 20 mg/Kg BB 8 – 12 jam SC 5 hari 5. Mikro Agregat / Mikro Emboli LOGO Paru, serebral, retina, renal 6. Emboli Udara Masuknya udara kedalam vena melalui tube transfusi. KESIMPULAN LOGO Transfusi darah transfusi komponen darah dari donor resipien Reaksi transfusi adalah komplikasi / efek samping yang terjadi akibat pemberian transfusi Reaksi transfusi dapat melalui proses imunologik/non imunologik Reaksi imunologi dapat terjadi cepat / lambat Manifestasi reaksi transfusi dapat menjadi fatal SARAN Kenali / waspadai adanya kemungkinan reaksi transfusi LOGO Materi 1. Pengertian dan Tujuan Transfusi 2. Golongan Darah 3. Tes Combs 4. Syarat-syarat atau Standar Pelayanan Transfusi 5. Pelayanan Permintaan darah 6. Reaksi Transfusi www.themegallery.com Company Logo TRANSFUSI DARAH LOGO Definisi Tujuan Suatu proses pemindahan 1. Mengatasi kekurangan volume darah darah dari orang sehat 2. Memperbaiki kemampuan transportasi kepada orang sakit oksigen 3. Memperbaiki pembekuan darah 4. Pengobatan seperti pada neutropenia berat Indikasi Transfusi Darah 1. Perdarahan 4. Anemia berat 2. Trombositopenia 5. Leukemia 3. Hemofilia 6. Hemolitic Desease of the Newborn (HDN) www.themegallery.com Company Logo Usaha-Usaha Memenuhi Kebutuhan Darah LOGO Meningkatkan jumlah donor ; Meningkatkan efisiensi penggunaan darah ; • kampanye 1. Memperkecil jml darah yg tdk terpakai ; • penerangan • Indikasi medik pemberian transfusi hrs tepat • motivasi , dll • Prosedur dan penyediaan darah hrs baik • Memperbaiki cara penyimpanan • Mencari pengawet baru yg dpt memperpanjang masa simpan darah • Mencari dan mengembangkan teknik-teknik baru. 2. Memisahkan komponen-komponen darah ; • Dari seorg dokter diberikan hanya yg bermanfaat bagi penderita. • Penderita tdk dibebani komponen yg tdk diperlukan, www.themegallery.com Company Logo Yang Perlu Dikerjakan di Laboratorium Transfusi Darah LOGO 1. Penentuan golongan 3. Reaksi Silang Darah ABO 4. Tes penyakit menular ; 2. Tentukan Tes Combs - Hepatitis B atau faktor Rhesus - Hepatitis C - HIV - Sifilis www.themegallery.com Company Logo Dari segi kedokteran transfusi darah tiada lain adalah suatu cara LOGO membantu pengobatan artinya transfusi darah tidak bisa berdiri sendiri atau hanya sebagai pelengkap dari metode pengobatan. Walaupun demikian transfusi darah itu bukanlah pekerjaan yang tanpa resiko dan bahkan dapat menambah penderita bagi si sakit, ini dikarenakan ; 1. Di bank darah tidak mungkin memeriksa semua macam golongan darah yg ada pada si sakit maupun si donor. misalnya mungkin gol.darah ABO dan Rhesus sama tapi belum tentu sistem gol.MN nya sama, kecuali kembar identik hanya bisa sama. Menurut penelitian hanya 1 antara 2000 org yg akan mempunyai gol.darah sama. 2. Kemampuan badan kita menolok, utk tdk menerima yg tdk cocok dengan badannya 3. Darah yang disimpan kurang baik, pengambilannya ceroboh shg kontak dengan kuman 4. Si donor menderita penyakit menular. Dari penelitian seorang dokter manfaat transfusi darah itu bukan untuk si pasien tapi untuk keluarganya karena keluarga merasa tenteram dgn segala pengobatan yang diberikan. Maka untuk kepada kita semualah tumpuan dan harapan untuk mengurangi penderitaannya. www.themegallery.com Company Logo LOGO www.themegallery.com Click to edit company slogan .