Erythrocyte Pyruvate Kinase Deficiency Resulting in Congenital Nonspherocytic Hemolytic Anemia

Abstract

HEREDITARY spherocytosis with its associated congenital hemolytic anemia is generally easily recognized and satisfactorily treated with splenectomy. A rarer group of congenital hemolytic anemias, however, is sometimes confused with hereditary spherocytosis because of the occasional presence of small numbers of spherocytes in association with varying degrees of abnormality in the incubated osmotic fragility test. In this later group of "congenital nonspherocytic hemolytic anemias" splenectomy has often been performed but usually does not produce a clinical cure. Because splenectomy is generally of inconsistent value, this type of anemia must be accurately differentiated for the sake of proper treatment and accurate prognosis. . . .