A rare case of coagulation disorder presenting with unexpected bleeding non-responding to fresh frozen plasma replacement: Factor XIII deficiency

Abstract

Factor XIII deficiency is a quite rare bleeding disorder in which detecting tests for coagulation are found in normal ranges. This disorder usually does not cause spontaneous bleeding but other than excessive bleeding after invasive procedures, impaired wound healing, abnormal scar formation, recurrent spontaneous miscarriages in women, oligospermia and infertility in men could be seen. Case Report: A thirty three years old male patient with previously diagnosed as factor XIII deficiency referred to emergency with serious stomachache which began after he fell down at his home. After recognition of a retroperitoneal hematoma and splenic rupture, laparotomic splenectomy with fresh frozen plasma (FFP) replacement was performed. Meanwhile factor XIII levels were detected <1%. Although FFP (15-20 ml/kg) and cryopresipitate was given postoperatively, bleeding from the incision and surgical drains continued. Until postoperative 15th day approximately 20 units of red blood cells were transfused. Because the bleeding couldn’t be stopped, plasma concentrate of factor XIII is brought from abroad and administered at a dose of 20 IU/kg (1500 U/day) weekly, for three weeks (totally three doses). After the administration of the first dose the amount of bleeding decreased progressively and eventually stopped. We reported this case because, besides factor XIII deficiency is a rare form of bleeding disorder; this patient had more severe and prolonged bleeding than expected and did not responded FFP or cryopresipitate therapy. So unexpected bleeding could not be stopped until plasma concentrate of factor XIII was administered, though to be worth for attention.