The following is a summary of “Natural history and rate of progression of retinopathy in adult patients with sickle cell disease: an 11-year follow-up study,” published in the July 2023 issue of Hematology by Brandsen, et al.
Sickle cell retinopathy (SCR) is a sickle cell disease (SCD) complication. Proliferative SCR (PSCR) is a more serious form of SCR that can lead to severe visual impairment.
Researchers performed a retrospective study to track the progression of SCR and determine factors that may likely worsen or lead to PSCR. They analyzed the progression of the disease in 129 patients with SCD over a median follow-up period of 11 years (interquartile range, 8.5-12), dividing patients into two groups. Patients with the genotypes hemoglobin SS (HbSS), HbSβ0-thalassemia, and HbSβ+-thalassemia were placed in 1 group (n = 83; 64.3%), whereas patients with HbSC were placed in a separate group.
Progression of SCR was observed in 28.7% of all patients, regardless of their genotype. Older patients( [aOR], 1.073; 95% [CI], 1.024-1.125; P= .003),(aOR, 25.472; 95% CI, 3.788-171.285; P ≤ 0.001) and lower HbF levels (aOR, 0.786; 95% CI, 0.623-0.993; P= .043) were more likely to develop PSCR at the end of follow-up. Patients who were female (aOR, 2.555; 95% CI, 1.101-5.931; P= .029) had HbSS/HbSβ0/HbSβ+ genotype (aOR, 3.733; 95% CI, 1.131-12.321; P= .031), and higher HbF levels (aOR, 1.119; 95% CI, 1.007-1.243; P= .037) were likely to develop any SCR at the end of follow-up.
The study concluded patients at low or high risk of SCR should be monitored differently.