Acquired Hemophilia First Manifesting as Life-Threatening Intracranial Hemorrhage

A 74-year-old man presented with life-threatening intracranial hemorrhage and prolonged activated partial thromboplastin time (APTT). The massive subdural hematoma was removed, but multiple intracranial hemorrhages occurred despite the administration of factor VIII and factor IX concentrates. Subdural hematoma, intracerebral hemorrhage in the left temporal lobe, and thalamic hemorrhage subsequently occurred with further prolongation of APTT. He died of enlargement of the thalamic hemorrhage. Acquired hemophilia was diagnosed caused by factor VIII inhibitor. Acquired hemophilia may cause life-threatening hemorrhage, and should be considered in patients with intracranial hemorrhages associated with unexplained prolongation of APTT.