Acquired Hemophilia First Manifesting as Life-Threatening Intracranial Hemorrhage -Case Report-

Abstract

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.

An epileptic seizure and haemorrhage into the ventricular system of the brain as the first manifestations of acquired haemophilia A – Case report

“…In 2009, Mashiko et al reported the case history of a 74-year-old man who was found comatose in his bed. CT revealed a massive subdural haematoma [10]. Among many laboratory evaluations only isolated prolonged APTT (77 seconds) was found.…”

Unusual Initial Manifestation of Acquired Hemophilia A: A Normal Activated Partial Thromboplastin Time, Intramuscular Hematoma and Cerebral Hemorrhage

“…However, if it occurs, it sometimes leads to death (7,8). In our patient, we detected intracranial hemorrhage and made a diagnosis of acquired hemophilia A before any life-threating events occurred.…”

“…Regarding the second clinical issue, most patients with acquired hemophilia A have hemorrhage in the skin, muscles or soft tissues ( 5 ), while intracranial hemorrhage is rare ( 6 ). However, if it occurs, it sometimes leads to death ( 7 , 8 ). In our patient, we detected intracranial hemorrhage and made a diagnosis of acquired hemophilia A before any life-threating events occurred.…”

Cerebellar hemorrhage as a first presentation of acquired Hemophilia A

“…A prolonged aPTT was present in all cases while further work-up of the laboratory abnormality was delayed in three of the four cases. Rebleeding with worsening of the clinical condition occurred in three of the cases, ultimately leading to death [4][5][6].…”

“…In 46.1% of the cases of AHA, no underlying disorder has been identified [3]. Intracranial hemorrhage has been reported in patients previously diagnosed with AHA [1,2]; however, intracranial hemorrhage as the presenting manifestation of AHA has only been described in three previous case reports [4][5][6]. Here, we present the case of an 86-year-old woman with an acute intraparenchymal cerebellar hemorrhage as the presenting manifestation of AHA.…”