Influence of athalassaemia on the retinopathy of homozygous sickle cell disease

Abstract

Homozygous α+ thalassaemia (α-/α-) ameliorates some of the clinical manifestations of homozygous sickle cell (SS) disease but its effect on retinal complications remains unknown. This has been assessed by visual examination and fluorescein angiography in 39 subjects with SS disease and homozygous α+ thalassaemia and in 39 age/sex matched controls with SS disease but with a normal α globin genotype (α α/ α α). The results indicate that homozygous α+ thalassaemia reduces the extent of peripheral retinal vessel closure but has no apparent effect on the frequency of proliferative sickle retinopathy.