Cerebral Thrombosis in /3-Thalassemia/Hemoglobin £ Disease

Thalassemia is a congenital hemolytic anemia prevalent in Asian and Mediterranean races. The homozygous state results in thalassemia major or Cooley's anemia, and such patients are transfusion-dependent. A less severe syndrome, thalassemia intermedia, may be due to compound heterozygosity for /3-thalassemia and /3 variant (e.g., hemoglobin E [HbE]) genes and is characterized by anemia, jaundice, hepatosplenomegaly, and delayed puberty. Patients with thalassemia intermedia require only occasional blood transfusions but often need splenectomy later in life because of progressive splenomegaly. Acute cerebrovascular insults are rarely reported in thalassemic patients. We describe two Chinese patients with /3-thalassemia/hemoglobin E (/Mhal/HbE) disease who developed cerebral infarction due to extracranial artery disease.