In an effort to identify possible risk factors for stroke in Sickle Cell Anemia (Hb SS), we analyzed the distribution of alpha‐globin gene deletions in a group of Hb SS patients with and without stroke. The group with stroke consisted of 44 patients, (27 male, 17 female) with a mean of 7.5 years at time of stroke. The control group (non‐stroke) had 256 Hb SS patients (126 male, 130 female) with a mean age of 7.7 years. There were 9 patients with heterozygous α‐thalassemia in the stroke group (20.5%). In the control group, there were 93 patients with heterozygous α‐thalassemia and 5 with homozygous α‐thalassemia. The incidence of α‐thalassemia in Hb SS patients without stroke (38%) was comparable to that reported for the African‐American population in general. The incidence in the stroke population (20.5%) was significantly lower (P = 0.02) These results indicate that α‐thalassemia is associated with a lower risk of stroke in Hb SS. This observation should be confirmed in studies involving larger numbers of patients. Possible protective effects of α‐thaiassemia are unknown but may be related to decreased hemolysis and more favorable rheologlc properties of red blood cells. © 1994 Wiley‐Liss, Inc.
- sickle cell disease
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