Clinical and Biochemical Interactions of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle-Cell Anemia

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Abstract
The extremely young red-cell population in sickle-cell anemia invariably results in increased G-6-PD activity. This effect is more marked in persons who genotypically have the sex-linked Gd A- type of G-6-PD deficiency, and may even mask the phenotypic expression of the enzyme deficiency. Electrophoresis and family studies were used to determine the genotype in patients with sickle-cell anemia. Among 15 such patients five were Gd A- as compared with 11 of 102 randomly selected Afro-American controls (0.05 < p > 0.01). These findings showing a relative preponderance of the Gd A- type in patients with sickle-cell anemia support the Lewis hypothesis that the simultaneous inheritance of both defects may be clinically beneficial.