Cerebellar hemorrhage as a first presentation of acquired Hemophilia A

• Published in: Neurocritical care Vol. 15; no. 1; pp. 170 - 174
• Main Authors: Micic, Dejan, Williams, Eliot C., Medow, Joshua E.
• Format: Journal Article
• Language: English
• Published: New York Humana Press Inc 01-08-2011; Springer Nature B.V
• Subjects: Aged, 80 and over; Anticoagulants; Blood pressure; Cerebral Hemorrhage - diagnosis; Cerebral Hemorrhage - etiology; Cerebral Hemorrhage - therapy; Critical Care Medicine; Female; Hematoma; Hemophilia; Hemophilia A - complications; Hemophilia A - diagnosis; Hemophilia A - therapy; Hemorrhage; Humans; Intensive; Internal Medicine; Laboratories; Medicine; Medicine & Public Health; Neurology; Patients; Practical Pearl; Surgery; Tomography; Acquired hemophilia A; aPTT; rFVIIa; Coagulation disorder; Intraparenchymal hemorrhage; FVIII; Lupus anticoagulant
• ISSN: 1541-6933; 1556-0961
• DOI: 10.1007/s12028-010-9489-0

Background
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.
Method
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.
Results
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 After discussion with the patient’s family, life-sustaining support measures were withdrawn.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.
Conclusion
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.