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reference materials

Management of Proliferative Sickle Cell Retinopathy

key information

source: American Academy of Ophthalmology

year: 2018

authors: Mashal Akhter, Michelle W. Latting, Adrienne W. Scott


Proliferative sickle cell retinopathy (PSR) is a vision-threaten-ing complication of sickle cell disease (SCD). Ischemic events in the retina stimulate angiogenesis, resulting in retinal neovascularization.

SCD is caused by a mutation in the HBB gene, which encodes hemoglobin beta. PSR affects up to 40% of hetero¬zygous (HbSC) patients and 20% of homozygous (HbSS) patients. Although most patients remain visually asymptomatic, the incidence of visual loss among patients with HbSS and HbSC affected by PSR has been reported as 31 per 1,000 eyes compared with 1.4 per 1,000 in eyes with nonproliferative disease.

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