Glucose 6-Phosphate Dehydrogenase Deficiency and Sickle Cell Anemia: Frequency and Features of the Association in an African Community

The glucose 6-phosphate dehydrogenase (G6PD) genotype was determined in 100 male patients with homozygous sickle cell anemia (55) by a combination of quantitative assay, cytochemical testing, and starch-gel electrophoresis. Of the 100 patients tested, 16 were found to be G6PD deficient (GdA_), and 84 G6PD normal (22 GsA and 62 Gd’). This distribution of G6PD genotypes did not differ significantly from that observed in the general population. The level of G6PD activity in GdA_ SS patients was nearly always higher than in G6PD-deficient subjects who did not have an associated hemolytic state, but it was nearly always lower than in G6PD-normal subjects. The clinical course of sickle cell disease, including the degree of anemia, was not milder in GdA_ than in G6PD-normal patients but could not be proved to be significantly more severe. It was concluded that in this community the incidence of G6PD deficiency in sickle cell anemia was not greater than would be expected by chance, and there was no evidence that the coexistence of the GdA_ gene in 55 patients ameliorated their disease.