Dairion Gatot, Soegiarto Ganie, Savita Handayani. Divisi Hematologi & Onkologi Medik Departemen Ilmu Penyakit Dalam FK-USU/RS H.Adam Malik Medan 2009

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1 HAEMOLYTIC ANAEMIA Dairion Gatot, Soegiarto Ganie, Savita Handayani. Divisi Hematologi & Onkologi Medik Departemen Ilmu Penyakit Dalam FK-USU/RS H.Adam Malik Medan 2009

2 WHEN BY THEN Hb 9 g% transfusion Hb 4 g% No sign of bleeding HEMOLYTIC?

3 Used Drug or traditional medicine Hb decreased no sign of bleeding Hemolytic?

4 Infection Anemia + icterus (mild) Hemolytic?

5 Definition Any situation in which there is a reduction in RBC life-span due to increase RBC destruction. Failure of compensatory marrow response results in anemia. Predominant site of RBC destruction is red pulp of the spleen.

6 Normositic normochromic anemia Diagnostic: MCV fl and MCH > 27 pg or MCHC ³ 30 g/dl Cause : Distribution failure. Lack of production, Rate of RBC destruction reduced red-cell life span

7 INTERPRETATION LABORATORY TEST PERIPHERAL SMEAR NORMOCYTIC ANAEMIA RETICULOCYTE COUNT INCREASED REDUCED BLOOD IN STOOL OR OTHER SOURCE OF BLEEDING IDENTIFIED POSITIVE NEGATIVE ANTIHUMAN GLOBULIN (COOMBS TEST) POSITIVE NEGATIVE NEGATIVE BONE MARROW AND BONE MARROW BIOPSY HYPERCELLULAR ERYTHROID HYPERPLASIA HYPERCELLULAR ERYTHROID HYPERPLASIA DECREASED CELLULARITY REPLACEMENT OF NORMAL MARROW ELEMENTS DIAGNOSIS BLOOD LOSS ANAEMIA AUTO IMMUNO HAEMOLYTIC ANAEMIA Erythroblastosis foetalis Transfusion reaction Collagen vascular disease OTHER HAEMOLYTIC ANAEMIAS Parasites Hypersplenism Microangiopathic haemolysis Hereditary spherocytosis Paroxysmal noctural haemoglobinuria OTHER Renal disease Infection Malnutrition Aplastic anaemia Radiation MYELOPHTHISIC Tumor Myelofibrosis Infection Leukaemia Enzyme deficiencies Drug or toxin Haemoglobinopathies

8 Classification of Hemolytic anemias I. Red cell abnormality (Intracorpuscular factors) A. Hereditary 1. Membrane defect (spherocytosis, elliptocytosis) 2. Metabolic defect (Glucoze-6-Phosphate Phosphate-Dehydrogenaze (G6PD) deficiency, Pyruvate kinase (PK) deficiency) 3. Hemoglobinopathies (unstable hemoglobins, thalassemias, sickle cell anemia ) B. Acquired 1. Membrane abnormality-paroxysmal nocturnal hemoglobinuria (PNH)

9 II. Extracorpuscular factors A. Immune hemolytic anemias 1. Autoimmune hemolytic anemia - caused by warm-reactive reactive antibodies - caused by cold-reactive antibodies 2. Transfusion of incompatible blood B. Nonimmune hemolytic anemias 1. Chemicals 2. Bacterial infections, parasitic infections (malaria), venons 3. Hemolysis due to physical trauma - hemolytic - uremic syndrome (HUS) - thrombotic thrombocytopenic purpura (TTP) - prosthetic heart valves 4. Hypersplenism

10 Mechanisms of hemolysis: - Intravascular - Extravascular

11 Intravascular hemolysis (1): - red cells destruction occurs in vascular space - clinical states associated with Intravascular hemolysis: acute hemolytic transfusion reactions severe and extensive burns paroxysmal nocturnal hemoglobinuria severe microangiopathic hemolysis physical trauma bacterial infections and parasitic infections (sepsis)

12 Inravascular hemolysis (2): - laboratory signs of intravascular hemolysis: indirect hyperbilirubinemia erythroid hyperplasia hemoglobinemia methemoalbuminemia hemoglobinuria absence or reduced of free serum haptoglobin hemosiderinuria

13 Extravascular hemolysis (1) - red cells destruction occurs in reticuloendothelial system - clinical states associated with extravascular hemolysis : autoimmune hemolysis delayed hemolytic transfusion reactions hemoglobinopathies hereditary spherocytosis hypersplenism hemolysis with liver disease

14 Extravascular hemolysis (2): Laboratory signs of extravascular hemolysis: indirect hyperbilirubinemia increased excretion of bilirubin by bile erythroid hyperplasia hemosiderosis

15 Autoimmune hemolytic anemia caused by warm- reactive antibodies: I. Primary II. Secondary 1. acute - viral infections - drugs ( α-methyldopa, Penicillin, Quinine, Quinidine) 2. chronic - rheumatoid arthritis, systemic lupus erythematosus - lymphoproliferative disorders (chronic lymphocytic leukemia, lymphomas, WaldenstrÖm s macroglobulinemia) - miscellaneous (thyroid disease, malignancy )

16 Autoimmune hemolytic anemia caused by cold-reactive antibodies: I. Primary cold agglutinin disease II. Secondary hemolysis: - mycoplasma infections - viral infections - lymphoproliferative disorders III. Paroxysmal cold hemoglobinuria

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