International Journal of Hematology

, Volume 95, Issue 4, pp 362–370 | Cite as

Factor XIII is a key molecule at the intersection of coagulation and fibrinolysis as well as inflammation and infection control

Progress in Hematology Current understanding of thrombosis and hemostasis—from bench to bedside

Abstract

Factor XIII (FXIII) is a transglutaminase consisting of two catalytic A subunits (FXIII-A) and two non-catalytic B subunits (FXIII-B) in plasma. FXIII-B protects FXIII-A from its clearance. FXIII-A is also present as a homodimer inside megakaryocytes/platelets and monocytes/macrophages. Although possible functions of intracellular FXIII-A have been proposed, these remain to be established. Intra- and extra-cellular FXIIIs support platelet adhesion and spreading as well as clot retraction, suggesting that FXIII is important for the stabilization of platelet–fibrin clots. Intra- and extra-cellular FXIIIs also support immobilization and killing of bacteria as well as phagocytosis by macrophages. Thus, FXIII may function in innate immunity. Congenital FXIII deficiency due to defective F13-A genes manifests as a life-long bleeding tendency, abnormal wound healing, and recurrent miscarriage. Although congenital FXIII-B deficiency used to be thought rare, reports of such cases have increased recently. As the bleeding tendency is often mild, patients with FXIII-B deficiency may be overlooked by physicians. Patients with acquired FXIII deficiency, in particular those with autoimmune hemorrhaphilia due to anti-FXIII antibodies, are on the increase, at least in Japan. It is important to diagnose such cases as early as possible, and to treat them with immunosuppression in combination with FXIII replacement therapy as their bleeding symptoms can be life-threatening.

Keywords

Plasma transglutaminase Fibrin-stabilizing factor Anti-fibrinolytic factor Innate immune factor Autoimmune hemorrhaphilia XIII/13 Hereditary factor XIII B subunit deficiency 

Supplementary material

12185_2012_1064_MOESM1_ESM.doc (162 kb)
Supplementary material 1 (DOC 162 kb)

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Copyright information

© The Japanese Society of Hematology 2012

Authors and Affiliations

  1. 1.Department of Molecular Patho-Biochemistry & -BiologyYamagata University School of MedicineYamagataJapan

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