Hemolytic anemias. Diagnosis and management.

Hemolysis can be induced by two general mechanisms. In the first one, erythrocytes lyse intravascularly due to complement fixation, trauma, or other extrinsic factors. In the second mechanism, which is the most common, the red cells are removed from the circulation by the mononuclear-phagocytic system either because they are intrinsically defective or because of the presence of bound immunoglobulins to their surfaces. The diagnosis of hemolysis is not difficult to establish and is based on the presence of anemia with sustained reticulocytosis in the absence of blood loss. Additional findings can include marrow erythroid hyperplasia; increased unconjugated bilirubin, LDH, and free hemoglobin; decreased haptoglobin and hemopexin; hemoglobinuria and hemosiderinuria; and decreased 51Cr red cell half-life. Hemoglobinemia, hemoglobinuria, and hemosiderinuria occur only in the setting of severe and rapid intravascular hemolysis. Conditions associated with significant lysis of red cells in the circulation include incompatible transfusion, G6PD deficiency, PNH, severe burns, and certain infections. The morphology of the red cell is abnormal in almost all cases of hemolytic anemia. However, the morphologic abnormality can be, in certain cases, diagnostic of the underlying condition. Treatment is usually supportive, with effective therapy directed to treat the underlying cause of hemolysis.