[Successful total gastrectomy of gastric cancer in a congenital factor V deficient patient]

Congenital factor V deficiency is a very rare hereditary coagulation disorder. Total gastrectomy in a patient with factor V deficiency has not been reported in Japan. A 71-year-old woman visited our hospital because of gastric cancer and gallbladder stone. A preoperative screening examination revealed severe anemia, prolonged prothrombin time (35.1 sec.) and activated partial thromboplastin time (109.8 sec.) The value of factor V was 8%. Her parents had a consanguineous marriage. The level of factor V in her two children and a grandchild were lower than the normal limit. We transfused fresh blood and fresh frozen plasma (FFP) preoperatively in order to improve anemia and prothrombin time and activated thromboplastin time. Operating carefully with transfused FFP and fresh blood, we performed total gastrectomy with cholecystectomy successfully. There was no serious tendency to hemorrhage during the operation and the postoperative period. Enough FFP should be transfused during the pre- and postoperative period, paying attention to pulmonary or cardiac failure in elderly patients. Postoperatively, during FFP should be used for 3-10 day with under careful observation of wound bleeding.