Korean J Hematol.  2002 May;37(2):164-167.

A Case of Congenital Factor XIII Deficiency Diagnosed by Semiquantitative Method

Affiliations
  • 1Department of Pediatrics, Dan Kook University College of Medicine, Chonan, Korea. pedpje@dankook.ac.kr
  • 2Department of Clinical Pathology, University of Ulsan, College of Medicine and Asan Medical Center, Seoul, Korea.

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.

Keyword

Factor XIII deficiency; Fibrin stabilizing factor; Semiquantitative method

MeSH Terms

Bleeding Time
Child
Factor XIII Deficiency*
Factor XIII*
Female
Fibrin
Hemorrhage
Humans
Intracranial Hemorrhages
Mass Screening
Plasma
Plasmapheresis
Polymers
Prothrombin Time
Reference Values
Thromboplastin
Tissue Donors
Factor XIII
Fibrin
Polymers
Thromboplastin
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