Newly Diagnosed Acquired Hemophilia A Manifesting as Massive Intracranial Hemorrhage Following a Neurosurgical Procedure.

Abstract

Increased attention has been paid to limiting preoperative hemostatic screening because assessment of patient history can be used as an alternative. However, there may be some clinical pitfalls in overlooking acquired coagulopathies. Here, we present a case of newly diagnosed acquired hemophilia A (AHA) that manifested as a massive intracranial hemorrhage without unexplained bleeding history or abnormal hemostatic results. A 58-year-old man, who had a history of surgical clipping of an anterior communicating artery aneurysm 30 years ago, experienced subarachnoid hemorrhage because of a ruptured middle cerebral artery aneurysm. He underwent surgical clipping and external decompressive craniectomy; 30 days later, cranioplasty was performed without preoperative hemostatic screening because of his normal coagulation status at the time of a previous surgery. Persistent wound bleeding and epistaxis suddenly began 6 hours after surgery. Computed tomography (CT) revealed a massive intracranial hematoma in the damaged parenchyma, although the patient was asymptomatic. At that time, laboratory tests showed isolated prolonged activated partial thromboplastin time and the presence of factor VIII inhibitor, which confirmed AHA. To manage the bleeding, fresh frozen plasma was transfused for 4 consecutive days, and hemostasis was finally achieved. Thereafter, the laboratory test results were normalized in 5 weeks. The patient's clinical course has been uneventful for 7 months without recurrence of AHA. Acquired coagulopathies are relatively rare but life-threatening. Because clinical history is insufficient to predict an acquired coagulopathy, preoperative hemostatic screening should be performed before each neurosurgical procedure.