Enzymatic Anomaly of Erythrocytes in Congenital Nonspherocytic Hemolytic Anemia

Abstract

Two families of congenital nonspherocytic hemolytic anemia were reported. A metabolic abnormality consisting of a low content of GSH, an abnormality in the GSH stability test and a low activity of G6PD in the erythrocytes from the patients was demonstrated. These abnormalities were also found in a milder degree in the erythrocytes from the mothers while there were no abnormalities in the erythrocytes from the fathers. An aggravation of hemolysis and metabolic disturbances mentioned above was observed following administration of Domian (6-sulfanilamido-2,4-dimethylpyrimi-dine) to the patient. It was also argued by way of experiment that the difference between the so-called congenital nonspherocytic hemolytic anemia and drug-induced hemolytic anemia is not essential, but only quantitative, so that both disorders probably belong to the same entity of enzymopathy from the viewpoint of pathogenesis of hemolysis.