Cerebrovascular accident in beta-thalassemia major (beta-TM) and beta-thalassemia intermedia (beta-TI).
Chronic hypercoagulable state expressed clinically by thromboembolic events has been described in thalassemia. One of the affected organs is the brain where symptomatic and asymptomatic damage has been reported. The present report describes seven cases who presented with the signs of cerebrovascular accident (CVA), five ischemic and two with hemorrhage. Two of them died. All patients were splenectomized, five received regular blood transfusions, and their ferritin levels were between 1,200 and 3,000 mg %. In addition, four patients had congestive heart failure and atrial fibrillation, and three had "Bronze diabetes," The recommendation on the basis of the results is that well-designed clinical trials are indicated to monitor asymptomatic brain damage by magnetic resonance imaging in splenectomized patients over the age of 20 years, who are not regularly transfused and have a high risk to develop thromboembolic events. In this subset of patients, anticoagulant and/or antiplatelet therapy should be considered. Moreover, treatment of additional complications resulting from iron overload, which may contribute to the etiology of CVA such as cardiac failure and arrhythmia with or without "bronze diabetes," is mandatory.
Karimi
Mehran
M
Department of Pediatrics, Thrombosis and Hemostasis Unit, Hematology Research Center, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
Khanlari
Mahsa
M
Khanlari
Maryam
M
Rachmilewitz
Eliezer A
EA
D000293:Adolescent; D000328:Adult; D001921:Brain / Q000098:blood supply*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D014057:Tomography, X-Ray Computed; D017086:beta-Thalassemia / Q000145:classification / Q000473:pathology*