Intracranial hemorrhage in acquired hemophilia A: illustrative case

Acquired hemophilia A (AHA) is a rare autoimmune coagulopathy caused by factor VIII inhibitors. Intracranial hemorrhage is an uncommon but potentially devastating presentation. The authors systematically reviewed reported cases of AHA-associated intracranial hemorrhage and present an illustrative case highlighting diagnostic and management considerations.

A PRISMA-guided review of PubMed, ScienceDirect, and Scopus included original case reports describing clinical features, management, or outcomes. Sixteen patients were identified, with a median age of 66.5 (IQR 15.5) years and slight male predominance (56%). Hemorrhage types included intracerebral hemorrhage (50%), acute subdural hematoma (38%), subarachnoid hemorrhage (12%), and intraventricular hemorrhage (19%); 3 patients had multiple hemorrhage types. Prolonged activated partial thromboplastin time was reported in 14 patients (mean 76.0 ± 28.6 seconds) with markedly reduced factor VIII activity. Hemostatic therapy was administered in 81% of patients and immunosuppression in 69%. Among those receiving hemostatic therapy, bypassing agents were used in 56% (recombinant activated factor VIIa 50%; activated prothrombin complex concentrate 19%). Surgical intervention occurred in 31%. The overall survival was 62%, and 30% of survivors achieved full neurological recovery. An additional case describes an 80-year-old woman with acute subdural hematoma who underwent decompressive craniectomy before AHA diagnosis and recovered after factor-directed and immunosuppressive therapies.

Intracranial hemorrhage in AHA is rare but highly morbid. Early recognition of isolated prolonged activated partial thromboplastin time and prompt initiation of bypassing agents may improve outcomes. https://thejns.org/doi/10.3171/CASE26195.