Enzymatic Anomaly of Erythrocytes in Congenital Nonspherocytic Hemolytic Anemia

Two families of congenital nonspherocytic hemolytic anemia were reported. A metabolic abnormality in the erythrocytes from the patients was demonstrated, consisting of a low content of GSH, an abnormality in the GSH stability test and a low activity of G6PD. These abnormalities were also found in the erythrocytes from the mothers in a milder degree, while there was 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-dimethylpyrimidine) 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 belong highly probably to the same entity of enzymopathy fromm the viewpoint of pathogenesis of hemolysis.