Biological impact of α genes, β haplotypes, and G6PD activity in sickle cell anemia at baseline and with hydroxyurea

Abstract

Key Points α genes and CAR haplotypes independently impact hemolytic anemia severity; low G6PD-activity impacts anemia severity in CAR/CAR patients. BEN/BEN patients have a higher prevalence of the favorable BCL11A/rs1427407 T allele and a better response to HU than CAR/CAR patients.