Abstract
Acquired hemophilia A is a rare, but refractory and life-threatening hemorrhagic disease, caused by an autoantibody to coagulation factor VIII. We report the case of an 80-year-old female without any history of abnormal bleeding who underwent esophagectomy for esophageal carcinoma. Two months after the operation she experienced the sudden onset of dyspnea associated with a right extrapleural hematoma and was transferred to our hospital. Her activated partial thromboplastin time was extremely prolonged, and there was continuous oozing from the venipuncture sites. Fresh-frozen plasma infusion was ineffective, and she was later diagnosed with acquired hemophilia A based on a positive test for autoantibody to factor VIII (36.0 Bethesda U·ml−1). We started treatment with prednisolone and recombinant activated factor VII, and the factor VIII autoantibody titer decreased to 1.2 Bethesda U·ml−1 by 70 days after admission. The patient experienced repeated nasal and digestive tract bleeding, but the bleeding and the size of extrapleural hematoma gradually decreased. The patient was discharged from the hospital 78 days after admission. The early diagnosis and rapid treatment would have contributed to her good outcome.
