발작성 야간혈색소뇨증에 동반된 뇌정맥혈전증 1예

Abstract

Paroxymal nocturnal hemoglobinuria is a kind of intravascular hemolytic anemia due to acquired corpscular defects, and is not rare in this country. The corpscular defect is originated from hematopoietic stem cells, and white blood cells and platelets are also involved. Consequently, the major complications which might affect mortality and morbidity include thrombotic phenomena, especially hepatic and cerebral venous thrombosis. This is a case report of cerebral venous thrombosis with hemiparesis, seizure and change of consciousness in paroxysmal nocturnal hemoglobinuria accompanied by intravascular hemolysis. The diagnosis is confirmed by CT brain scan and cerebral angiography and the patient is effectively treated with antiplatelets, anticonvulsants and steroids.