A Case of Acquired Hemophilia A after Surgery for Congenital Biliary Dilatation

Abstract

A 57-year-old woman was hospitalized because of abdominal pain and diagnosed with congenital biliary dilatation. She had no anemia or abnormal coagulation on admission. Elective extrahepatic biliary resection was performed with blood loss of 759 ml, without blood transfusion. On postoperative day (POD) 1, a slight decrease of the activated partial thromboplastin time (APTT) was observed. On POD 3, rapid progression of anemia was observed, and CT showed intra-abdominal hemorrhage. At reoperation, a large intra-abdominal hematoma was observed, but the bleeding point was unclear. On POD 4, further reoperation was needed for rebleeding, with the same result. Acquired hemophilia was suspected from the decrease in coagulation factor VIII and the result of the APTT mixing test. Later, factor VIII inhibitor was detected, and the diagnosis of acquired hemophilia A was confirmed. The treatment was difficult because of massive bleeding from the surgical sites, including gastrointestinal bleeding. In addition, serious wound separation and an intestinal fistula made it even more difficult. Remission of hemophilia was achieved with great effort, with the help of negative wound pressure therapy and endoscopic hemostasis.