Hemolytic Anemia 20161028.pptVIP

Hemoglobinopathies---Hemoglubin electrophoresis PNH positive Ham’s test (acid hemolysis test) positive sugar-water test specific immunophenotype of erytrocytes (CD59, CD55) Diagnosis Is there hemolysis? Look for evidence of red cells destruction Direct evidence Radioactive chormium(51Cr) labelling of red cells measures lifespan Indirect evidence Hemogoblin level decreased red cell fragments (microangiopathic anemias increased unconjugated bilirubin increased lactate dehydrogenase Is there hemolysis? Look for evidence of Marrow response to hemolysis Reticulocytosis Erythroid hyperplasia in marrow There is a circulating nucleated red blood cell (NRBC) There are nuclear remnants in RBC,such as Howell-Jolly bodies and Cabot rings Extravascular vs Intravascular hemolysis Extravascular Intravascular Test Hemolysis Hemolysis LDH á áá bilirubin á á haptoglobin N to absent absent hemoglobinuria absent present free Hb in plasma absent present urine hemosiderin absent present Laboratory studies associate with special HA Autoimmune hemolytic anemia Coomb’s test Hereditary spherocytosis Peripheral blood film erythrocyte osmotic fragility test G-6-PD deficiency G-6-PD activity test methemoglobin reduction test (+) Heinz bodies Laboratory studies associate with special HA Hemoglobinopathies Hemoglubin electrophoresis PNH positive Ham’s test (acid hemolysis test) positive sugar-water test specific immunophenotype of erytrocytes (CD59,  CD55) Treatment Treatment Therapeutic strategies for hemolytic anemia are determined by the underlying cause of red cell destruction, the magnitude of the anemia, and cardiopulmonary status of the patient. Treatment For extrinsic causes, the treatment plan usually becomes obvious at the time of diagnosis. Immune-mediated hemolysis may require immunoglobulin infusion, corticosteroids, or other immunosuppressive therapies. Prevention of immune- or G6PD-mediated hemoly