title: 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.
authors.affiliation: Surgery for Organ Function and Biological Regulation, Graduate School of Medicine, Nippon Medical School, Japan. ishi-1@nms. ac.jp
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Ishikawa
authors.forename: Kiyonori
authors.initials: K
authors.lastname: Furukawa
authors.forename: Kimiyoshi
authors.initials: K
authors.lastname: Yokoi
authors.forename: Tomoko
authors.initials: T
authors.lastname: Seya
authors.forename: Koji
authors.initials: K
authors.lastname: Horiba
authors.forename: Noritake
authors.initials: N
authors.lastname: Tanaka
authors.forename: Takashi
authors.initials: T
authors.lastname: Tajiri
mesh_terms: D000230:Adenocarcinoma / Q000601:surgery*; D014670:Ampulla of Vater; D015415:Biomarkers / Q000097:blood; D003138:Common Bile Duct Neoplasms / Q000601:surgery*; D005169:Factor VIII / Q000008:administration & dosage / Q000032:analysis; D006467:Hemophilia A / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D033162:Incidental Findings; D008297:Male; D008875:Middle Aged; D016577:Pancreaticoduodenectomy; D010314:Partial Thromboplastin Time; D019106:Postoperative Hemorrhage / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D005169:Factor VIII