Clinical and laboratory risk factors for sickle cell retinopathy and maculopathy: a scoping review of the current evidence
Rajani P. Brandsen,
Roselie M.H. Diederen,
Gizem Kocabas,
Erfan Nur,
Arjan Malekzadeh,
Reinier O. Schlingemann,
Bart J. Biemond
Affiliations
Rajani P. Brandsen
Department of Ophthalmology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands; Department of Hematology, Amsterdam UMC, University of Amsterdam, Amsterdam
Roselie M.H. Diederen
Department of Ophthalmology, Amsterdam UMC, University of Amsterdam, Amsterdam
Gizem Kocabas
Department of Ophthalmology, Amsterdam UMC, University of Amsterdam, Amsterdam
Erfan Nur
Department of Hematology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands; Sanquin Research and Landsteiner Laboratory, Department of Blood Cell Research, Amsterdam
Arjan Malekzadeh
Medical Library, Amsterdam UMC, University of Amsterdam, Amsterdam
Reinier O. Schlingemann
Department of Ophthalmology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands; Department of Ophthalmology, University of Lausanne, Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, Lausanne
Bart J. Biemond
Department of Hematology, Amsterdam UMC, University of Amsterdam, Amsterdam
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 hematological risk factors. SCR risk factors include older age, male sex, HbSC genotype, hemolysis and HbF% 15%) patients to ensure comprehensive yet proportionate ophthalmic care. However, to elucidate the sense and nonsense of screening, future studies are needed on the role of interventions for SCR and the long-term consequences of SCM.
