Practical Pearl

Neurocritical Care

, Volume 15, Issue 1, pp 170-174

First online:

Cerebellar hemorrhage as a first presentation of acquired Hemophilia A

  • Dejan MicicAffiliated withUniversity of Wisconsin School of Medicine and Public Health
  • , Eliot C. WilliamsAffiliated withDepartment of Internal Medicine, Section of Hematology, University of Wisconsin School of Medicine and Public Health
  • , Joshua E. MedowAffiliated withDepartment of Neurosurgery, University of Wisconsin School of Medicine and Public Health Email author 

Rent the article at a discount

Rent now

* Final gross prices may vary according to local VAT.

Get Access



Acquired hemophilia A (AHA) is an uncommon coagulation disorder caused by the development of autoantibodies against coagulation factor VIII (FVIII). While intracranial hemorrhage is a known complication of AHA, intracranial hemorrhage as the presenting manifestation of AHA has only been described in three previous case reports.


We report a case of an 86-year-old woman with no previously reported history of coagulopathy presenting with an acute intraparenchymal cerebellar hemorrhage and laboratory studies demonstrating an isolated prolonged activated partial thromboplastin time (aPTT). We discuss an approach to the prolonged aPTT, and review the literature concerning the diagnosis and treatment of AHA.


Occipital decompressive craniectomy with evacuation of the hemorrhage was performed. Eight hours following the procedure, the patient’s status acutely declined with demonstration of a reoccurrence of the cerebellar hemorrhage and new right frontal lobe hemorrhage. After discussion with the patient’s family, life-sustaining support measures were withdrawn. Postmortem analysis revealed a low FVIII activity level and the presence of FVIII inhibitor.


The presentation of intracranial hemorrhage with an isolated prolonged aPTT is concerning for an acquired hemophilia with FVIII deficiency. Other causes of isolated prolonged aPTT such as a lupus anticoagulant must also be considered. Preoperative identification and work-up of the coagulation abnormality is essential to guide initial treatment.


Acquired hemophilia A Coagulation disorder aPTT Intraparenchymal hemorrhage FVIII rFVIIa Lupus anticoagulant