ACTIVITY OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE IN ERYTHROCYTES OF PATIENTS WITH VARIOUS ABNORMAL HEMOGLOBINS

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Abstract
In 99 patients with sickle cell anemia and 100 normal Negro children, the activity of glucose-6-phosphate dehydrogenase in erythrocytes was determined. Hemoglobinopathies may be combined with reduced activity of glucose-6-phosphate dehydrogenase in the erythrocytes. The two defects are inherited independently. The incidence of the enzymatic defect in the erythrocytes of those with sickle cell anemia in this series does not differ from that of a similar Negro population having normal hemoglobin. There appears to be no significant effect of the enzymatic defect in the erythrocytes on the clinical course of patients with sickle cell anemia or survival times of their erythrocytes. Patients with sickle cell anemia and with normal stability of the glutathione in the erythrocytes have significantly higher levels of G6PD than controls or patients with sickle cell trait.

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