BACKGROUND: 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. OBSERVATIONS: 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. LESSONS: 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.
Witten et al. (Mon,) studied this question.