A man with beta thalassemia / hemoglobin E complicated by multiple cerebral venous sinus thromboses: A case report

Abstract

One of rare complications of beta thalassemia/hemoglobin E is the venous thrombosis particularly the cerebral venous thrombosis. Herein, we reported the case of a 28-year-old Thai man presenting with a chronic headache for a month and weakness, rightupper limb numbness for one day and tonic clonic convulsion for 15 minutes. Since early childhood, he had been diagnosed as severe beta thalassemia/Hb E. Splenectomy was performed at 8 years of age. He was regularly transfused around 6 units a year and he took oxymetholone for stimulation of erythropoiesis, and oral contraceptive pills for the future sexual conversion, without aspirin. The physical examination revealed typical thalassemicfacy, pallor, huge hepatomegaly, the motor power grade III at the rightupper limb. The blood tests were: Hb 7.2 g%, WBC 54,800/mm³, platelet 864,000/mm³, NRBC 200/100 WBC, protein S 51.7 %. The CTA, MRI and MRA of the brain showed subacute thromboses of the left cortical vein, superior sagittal sinus and right sigmoid sinus and subarachnoid hemorrhage at the left central and postcentral sulci.He fully recovered within a week after the treatment with enoxaparin, warfarin and phenytoin. The factors that might contribute the formation of cerebral venous sinus thromboses in our case included the oral pill, androgen, splenectomy, low protein S, thrombocytosis and numerousnucleated red blood cells in the peripheral blood.