Diagnosis

The anemia is usually normocytic and normochromic, and its severity ranges from moderate to extreme. Acute hemolytic anemia is due largely to intravascular hemolysis, and hence is associated with hemoglobinemia and hemoglobinuria. The blood film may show spectacular evidence of hemolysis in the guise of anisocytosis, polychromasia, spherocytes, bite cells, blister cells and hemighosts. Supravital staining reveals the presence of Heinz bodies, consisting of precipitates of denatured hemoglobin. In CNSHA the morphology is less characteristic. The final diagnosis must rely on the direct demonstration of decreased activity of G6PD in red cells by an appropriate enzyme assay.

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