CEREBROVASCULAR ACCIDENTS IN SICKLE-CELL DISEASE - RATES AND RISK-FACTORS

Cerebrovascular accident (CVA) is a major complication of sickle cell disease, The incidence and mortality of and risk factors for CVA in si ckle cell disease patients in the United States have been reported onl y in small patient samples. The Cooperative Study of Sickle Cell Disea se collected clinical data on 4,082 sickle cell disease patients enrol led from 1978 to 1988. Patients were followed for an average of 5.2 +/ - 2.0 years, Age-specific prevalence and incidence rates of CVA in pat ients with the common genotypes of sickle cell disease were determined , and the effects of hematologic and clinical events on the risk of CV A were analyzed, The highest rates of prevalence of CVA (4.01%) and in cidence (0.61 per 100 patient-years) were in sickle cell anemia (SS) p atients, but CVA occurred in all common genotypes, The incidence of in farctive CVA was lowest in SS patients 20 to 29 years of age and highe r in children and older patients, Conversely, the incidence of hemorrh agic stroke in SS patients was highest among patients aged 20 to 29 ye ars, Across all ages the mortality rate was 26% in the 2 weeks after h emorrhagic stroke. No deaths occurred after infarctive stroke, Risk fa ctors for infarctive stroke included prior transient ischemic attack, low steady-state hemoglobin concentration and rate of and recent episo de of acute chest syndrome, and elevated systolic blood pressure, Hemo rrhagic stroke was associated with low steady-state hemoglobin and hig h leukocyte count, (C) 1998 by The American Society of Hematology.