Open access journal of the Ferrata-Storti Foundation, a non-profit organization Open access journal of the Ferrata-Storti Foundation, a non-profit organization Department of Internal Medicine H, Rambam Health Care Campus, Haifa Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa Section of Clinical Pharmacology and Toxicology, Rambam Health Care Campus, Haifa, Israel; The Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa Department of Hematology and Bone

Open access journal of the Ferrata-Storti Foundation, a non-profit organization Open access journal of the Ferrata-Storti Foundation, a n on-profit organization Department of Medicine, University of Verona and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy; Veneto Region Referral Center for Iron Disorders and European Reference Network Center for Rare Hematological Diseases “EuroBloodNet “Department of Medicine, University of Verona and Azienda Ospedaliera Universitaria Integrata of

Sickle cell retinopathy (SCR) is a complication of sickle cell disease (SCD) and can drastically impair visual acuity. Screening for SCR is, therefore, recommended, but evidence for optimal screening frequency on an individual level is lacking. This scoping review mapped the current evidence on risk factors for SCR and sickle cell maculopathy (SCM). A literature search (in Medline [Ovid]), Embase [Ovid]), and Scopus) resulted in 67 included articles which covered demographic risk factors, genetic risk factors, systemic therapy, correlations with other forms of SCD-related organ damage, and hematologic risk factors. SCR risk factors include older age, male sex, HbSC genotype, hemolysis, and HbF% <15% (in HbSS) and increased blood viscosity (in HbSC). For SCM, risk factors are older age, HbSS genotype, and higher degree of hemolysis. The pathophysiology of SCR and SCM appears multifactorial, but distinct patterns emerge suggesting that vaso-occlusion and hemolysis cause SCM and NPSCR in