A Case of Congenital Factor XIII Deficiency Diagnosed by Semiquantitative Method / 대한혈액학회지

ABSTRACT

Factor XIII (fibrin stabilizing factor) has roles of stabilizing clot and cross-linking fibrin polymer, so the congenital factor XIII deficient patient has clot unstability and delayed bleeding episodes. We experienced a case of 11 years old girl who had experienced delayed umbilical healing, several episodes of intraabdominal and intracranial hemorrhage. But her coagulation screening studies with prothrombin time, aPTT (activated partial thromboplastin time), bleeding time showed normal value at each episode. These findings suggested typical features of congenital factor XIII deficiency. We used semiquantitative method to diagnose Factor XIII deficiency and quantify Factor XIII. We has treated her successfully with prophylactic fresh frozen plasma through plasmapheresis from two donors.