Abstract
Congenital factor XI deficiency is a rare hereditory coagulation disorder which was first reported by Rosenthal and his coworkers in 1953. Only forty-old families of the disease have been reported in Japan, but operated cases in such patients were very few. In this paper a successful gastrectomy for gastric cancer in a patient with this hereditory disease is described.
A 70-year-old man with factor XI deficiency (factor XI activity: less than 1%), who did not show bleeding tendency preoperatively, underwent subtotal gastrectomy for gastric cancer. Before surgery fresh frozen plasma and fresh blood (about 25ml/kg) was supplied to maintain intraoperative factor XI activity at more than 50%, and subtotal gastrectomy with node dissection was performed. For postoperative 7 days transfusion with fresh frozen plasma (5ml/kg) was given to maintain the activity at about 30%. This replacement therapy saved the patient from intra- and postoperative bleeding tendency and postoperative complications.
