Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting millions of people worldwide, particularly in populations of African, Latin American, and Mediterranean descent. Among its most severe complications is stroke, which can be ischemic or hemorrhagic, and is one of the leading causes of morbidity and mortality in individuals with SCD, particularly children and adolescents. The pathophysiology of stroke in SCD involves complex mechanisms such as vaso-occlusion, endothelial dysfunction, chronic anemia, and hypercoagulability. Despite its clinical relevance, epidemiological indicators of stroke in this population remain poorly explored in population-based studies in Brazil. Understanding these indicators is essential to guide prevention strategies, early diagnosis, and appropriate clinical management.

To assess the prevalence, incidence, and recurrence of ischemic and hemorrhagic stroke in patients with SCD, according to sex, age group, center, and genotype.

Cohort study including 2,793 SCD patients participating in the Recipient Epidemiology and Donor Evaluation Study-III (REDS-III) project, conducted in six Brazilian blood centers: Belo Horizonte, Montes Claros, Juiz de Fora, São Paulo, Rio de Janeiro, and Recife. Prevalence, incidence, and recurrence rates of stroke were reported as percentages with corresponding 95% confidence intervals (95% CI).

For ischemic stroke, there were no significant differences between females and males in prevalence (7.30% vs. 6.02%), incidence (0.95% vs. 0.46%), or recurrence (0.95% vs. 1.07%). By age group, prevalence was higher in adults compared to children (8.30% vs. 5.34%), with no significant differences in incidence (1.02% vs. 0.46%) or recurrence (0.94% vs. 1.06%). Regarding centers, the highest prevalence rates were observed in Recife (8.55%) and Rio de Janeiro (8.90%); the highest incidence rates in Belo Horizonte (1.02%) and Juiz de Fora (1.09%); and the highest recurrence rate in Montes Claros (3.03%). In genotype analysis, the highest prevalence was found in SS patients (8.80%), the highest incidence in SB⁰ patients (2.30%), and the highest recurrence rate in SS patients (1.37%). For hemorrhagic stroke, 17 prevalent cases were identified, 16 of which occurred in patients with the SS genotype. There were 5 incident cases and only 1 recurrent case, all in patients with a prior history of ischemic stroke.

This study showed that ischemic stroke is a frequent and recurrent complication in patients with sickle cell disease, with higher prevalence among adults and those with the SS genotype. Hemorrhagic stroke was less common but concentrated in patients with a history of ischemic stroke, suggesting possible clinical progression. The variations observed across centers and genotypes reinforce the importance of targeted surveillance and prevention strategies, particularly for high-risk groups. These findings contribute to improving care policies and clinical management of sickle cell disease in Brazil.