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Sickle Cell Disease (SCD) is an inherited disorder resulting in production of Haemoglobin S (HbS), which aggregates in conditions of hypoxia, acidosis or hyperosmolarity. This leads to vascular stasis, thrombosis and ischaemia. The authors present a cross-sectional study (45 consecutive patients) aiming to identify clinical, laboratory and / or ophthalmological predictors of proliferative sickle cell retinopathy (PSR) to define a screening strategy to identify patients at a higher risk of developing eye complications. The patients were split into two groups, those with non-proliferative retinopathy (NPSR) and those with PSR. Sex, age, best corrected visual acuity (BCVA), intraocular pressure (IOP) and cup-to-disc ratio (CDR) did not differ between the two groups. mean corpuscular volume (MSV), lactate dehydrogenase (LDH) and percentage of foetal haemoglobin (HbF) were reduced in the patients with PSR compared to NPSR. The best predictor of PSR was MSV, followed by HbF and LDH. The size of Foveal Avascular Zone (FAZ) on OCTA were similar in both groups. The authors postulate that these associations indicate that hypoxia, not haemolysis, is a possible driving force in sickle cell retinopathy (SCR) pathophysiology. This study proposes MSC and HbF as possible biomarkers to be used for screening for PSR. Further studies on red blood cell interaction with endothelial cells are advised.

Identifying clinical predictors of proliferative sickle cell retinopathy.
Serras-Pereira R, Vieira L, Saunders CJ, et al.
CURRENT EYE RESEARCH
2023;48(11):1063–7.
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Kurt Spiteri Cornish

Sheffield Teaching Hospitals NHS Trust, London, UK.

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