Fatal Pulmonary Thromboembolism in a Patient with Paroxysmal Nocturnal Hemoglobinuria

Abstract

A 38 year old woman with paroxysmal nocturnal hemoglobinuria was admited to our hospital because of hemoptysis. Pulmonary infarction was diagnosed by the perfusion lung scan. In spite of the administration of prednisolone, dextran, low molecular weight heparin, and warfarin, she died of pulmonary infarction and secondary pulmonary hypertension. Autopsy revealed thromboembolism of both pulmonary arteries and hepatic central vein thrombosis. Recent understanding of the pathogenesis and incidence of thromboembolism in paroxysmal nocturnal hemoglobinuria was discussed.