The role of hydroxyurea to prevent silent stroke in sickle cell disease

Abstract

Background: Chronic blood transfusions are standard of care for stroke prevention in sickle cell disease but is not cost effective and not without risks. Hydroxyurea has emerged as an option in the prevention of silent stroke in sickle cell disease. Objective: To evaluate the role of hydroxyurea in preventing silent strokes in a systematic review by adhering to the Cochrane guidelines. Methods: PubMed, EMBASE, Web of Science Core Collection, and Cochrane Central Register of Controlled Trials were searched for the related articles. Eligibility criteria included randomized controlled trials (RCTs) comparing the use of hydroxyurea vs blood transfusions and observational studies evaluating the role of hydroxyurea to prevent stroke and silent stroke in patients with sickle cell anemia or sickle cell β thalassemia. The meta-analysis was conducted using STATA software version 13. Results: We included 10 single arm observational studies with 361 participants, and one RCT study with 60 participants receiving hydroxyurea, respectively. There were no deaths attributed to hydroxyurea. The results revealed that 1% (95% CIs 0.0 to 0.05) of patients receiving hydroxyurea had stroke. 18% (95% CIs 0.03 to 0.4) of the hydroxyurea patients had silent stroke. 24% (95% CIs 0.02 to 0.57) of the hydroxyurea patients had adverse events attributed to hydroxyurea. Conclusion: Our findings suggest that hydroxyurea is safe and may prevent silent stroke and stroke in sickle cell disease. More high-quality studies including RCTs are needed.

Influence of Haptoglobin Polymorphism on Stroke in Sickle Cell Disease Patients

“…These occur in children with SCD younger than age 6 and increase in occurrence with age. A study showed that 27% of children with SCD younger than age 6 experienced SCI and by the age of 14, 37% of children with SCD experienced SCI [ 40 ]. A similar study in an adult population of patients with SCD with a relatively low mean age of 30 years showed that 53% had brain lesions consistent with SCI [ 41 ].…”

“…Although these two treatments have been shown to improve chronic and acute SCD, both require strict adherence, limiting the scope of patients who can be treated via these methods. In addition to the recommended routine adherence, regular blood exchange transfusions are costly and can put patients with SCD at further risk for disease progression by causing iron overload [ 22 , 40 ]. ROS are produced by excess iron via the Fenton reaction, exacerbating SCD symptoms of blood component toxicity [ 7 ].…”

Cerebral Vasculopathy in Children With Sickle Cell Disease in an Amazonian Population

“…Folic acid in association with acetylsalicylic acid was used in 2 patients (16.6%). A systematic review conducted by Hasson et al 23 found insufficient evidence to prove hydroxyurea's ability to prevent stroke in patients with sickle cell disease. However, hydroxyurea has been shown to be a viable option, because it is well-tolerated, lower cost, and has fewer side effects relative to regular blood transfusion.…”

Anemia falciforme y la resistencia a la malaria. Revisión narrativa

“…Las transfusiones crónicas de componentes sanguíneos se consideran el estándar de cuidado para la prevención de accidentes cerebrovasculares, a pesar de presentar riesgos como la sobrecarga de hierro y la aloinmunización (39,40). Teniendo en cuenta los riesgos y la costoefectividad se prefieren alternativas como la hidroxiurea, la cual parece ser segura y efectiva.…”