Background: Overt stroke in sickle cell anemia (SCA) is associated with intracranial stenosis and thrombus formation. Platelet activation is critical for thrombus formation. Procedure: Platelet activation studies were performed in 50 subjects: 18 SCA patients with history of stroke or abnormal transcranial Doppler (TCD) and intracranial stenosis seen by magnetic resonance angiogram (MRA), 7 SCA patients with history of stroke or abnormal TCD but no intracranial stenosis, 13 SCA patients with no history of stroke or abnormal TCD, and 12 healthy African-Americans. Results: Of the 18 patients with intracranial stenosis, 11 (61%) had evidence of the moyo-moya phenomenon on MRA. SCA children with intracranial stenosis had a significantly greater total white cell count compared to both healthy African-American controls and SCA patients in the steady-state (P<0.001). In addition, SCA patients with history of stroke or abnormal TCD had a significantly higher platelet count compared to healthy African-American controls (P<0.002). The percentage of platelet surface P-selectin expression was significantly greater in patients with intracranial stenosis compared to the other groups (P<0.05), particularly in individuals that did not have the moya-moya phenomenon seen on MRA. Conclusion: Stroke with intracranial stenosis is associated with increased platelet activation in sickle cell anemia, and further investigation is needed on the role of anti-platelet agents in this high-risk population.
- Sickle cell anemia
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health