Management of a Patient with Factor XI Deficiency in Total Hip Joint Replacement by Plasma Exchange Transfusion

Factor XI deficiency is a rare hereditary coagulation disorder and mostly its bleeding manifestation is excessive bleeding after surgery and trauma. Hemostasis of a 69-year-old Japanese woman with factor XI deficiency in total hip joint replacement was successfully achieved by plasma replacement therapy. Plasma exchange was took place preoperatively with 2800ml of fresh frozen plasma. Factor XI level of the patient plasma showed 65% of normal immediately after the plasma exchange and 57% at the operation. Subsequently, fresh frozen plasmas were transfused to maintain factor XI level at 40% for 24 hours after surgery then 30% for the next 3 days. Factor XI level and APTT were monitored and no excessive bleeding was observed during and after the operation.