Perioperative management of factor XI deficiency in a patient undergoing hip arthroplasty.

Factor XI deficiency, or hemophilia C, is a rare autosomal recessive bleeding disorder often diagnosed by inappropriate bleeding associated with trauma or a surgical procedure, and reports of anesthetic management of this disorder are rare. We experienced an 85-year-old man with femoral neck fracture who was diagnosed preoperatively with factor XI deficiency based on abnormally long activated partial thromboplastin time (APTT). He was scheduled for bipolar hip arthroplasty and was prepared for surgery by transfusion of fresh frozen plasma (FFP), instead of factor XI concentrates, which are not commercially available in Japan. Five units of FFP were transfused 6 days before surgery, and 10 units of FFP with 2 units of red concentrated cells (RCC) were used on the day of surgery. Transfusion of FFP shortened the APTT to a level sufficient to allow hemostasis, although not to within the normal range. Although the patient required transfusion of 2 units of RCC postoperatively, no bleeding complications occurred. For bipolar hip arthroplasty, transfusion of FFP produced sufficient hemostasis without the use of tranexamic acid, factor VII preparations, or desmopressin.