Alpha thalassemia and stroke risk in sickle cell anemia

American Journal of Hematology - Tập 45 Số 4 - Trang 279-282 - 1994
Robert Adams1, Abdullah Kutlar2,3, Virgil McKie4, Elizabeth Carl1, Fenwick T. Nichols1, Jin Cal Liu3, Kathleen T. McKie1, A.R. Clary1
1Department of Neurology, Medical College of Georgia, Augusta
2Department of Medicine, Medical College of Georgia, Augusta
3Departments of Biochemistry and Molecular Biology, Medical College of Georgia, Augusta
4Departments of Pediatric Hematology/Oncology, Medical College of Georgia, Augusta

Tóm tắt

AbstractIn 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.

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