Mild Hemophilia A Diagnosed in a 55-year-old Patient after Pancreatoduodenectomy for Carcinoma of the Papilla of Vater

Abstract

Hemophilia A is a sex-linked hereditary disease, and the total number of patients with this condition is small. It is quite rare for general surgeons to encounter a patient with hemophilia A. Moreover, it is extremely rare for surgeons to encounter adult patients with undiagnosed hemophilia. We describe a patient in whom intra-abdominal bleeding persisted after open abdominal surgery, leading to a diagnosis of hemophilia A. The patient was a 55-year-old man with carcinoma of the papilla of Vater who underwent pancreatoduodenectomy, during and after which hemostatic difficulties were encountered. Our initial diagnosis was complex coagulopathy; however, transfusion of a large volume of fresh frozen plasma did not improve the activated partial thromboplastin time, which led us to suspect hemophilia. Thorough personal and family histories and determination of coagulation factor VIII showed that the patient belonged to a family with hemophilia A, which had not been recognized by his parents, leading to a diagnosis of mild hemophilia A based on decreased coagulation factor VIII levels. After diagnosis, intermittent administration of a coagulation factor VIII product controlled the bleeding. The patient is currently being treated on an outpatient basis and remains free of cancer recurrence.