Sickle Cell Disease and the Eye: Old and New Concepts

Abstract 

The pathophysiology of sickle cell disease is not limited to abnormal red blood cells. The clinical manifestations of sickle cell disease include complex pathways and processes such as endothelial activation, inflammation, bioavailability of nitric oxide, oxidative stress, and the adhesiveness of a variety of blood cells. Increasingly, distinct subphenotypes and genetic modifiers of sickle cell disease are being recognized. We apply recent advances in sickle cell disease to ocular biology to highlight translational research in this field and encourage additional studies on the ocular manifestations of sickle cell disease.

Key words: animal studies, genetic modifiers, ocular manifestations, proliferative sickle retinopathy, sickle cell disease, sickle cell retinopathy