Replacement therapy in a patient with congenital prekallikrein deficiency undergoing surgery

A patient with congenital prekallikrein (PK) deficiency underwent left endonasal radical ethmoidectomy and nasal polypectomy after receiving a transfusion of 6 ml/kg body weight of fresh frozen plasma (FFP). His plasma PK activity was under 1% before transfusion, and 46% after transfusion. The patient had no bleeding tendency during operation, but bled extensively from the wound 7 days after operation. At that time, his plasma PK activity was 3%. Since the bleeding could not be stopped simply by plugging his nasal cavities with tampons, he was given 3 ml/kg body weight of FFP. As a result, his nasal bleeding rapidly decreased. No clinical sign of thrombotic complication was observed throughout the clinical course.