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All About Bleeding

Factor XIII Deficiency

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The Coagulation Cascade

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Introduction

Factor XIII (FXIII, also called fibrin stabilization factor) is a coagulation factor (a transglutaminase) that can be found in plasma, thrombocytes and monocytes as well as in different tissues (placenta, uterus and prostate).28 It consists of subunits A and B. Subunit A is the catalytic unit and subunit B is the carrier unit. In plasma it circulates as a heterotetramer. When a clot forms in response to blood vessel injury, bleeding is controlled via the coagulation process. In the final phase of coagulation, factor XIII is activated by thrombin and calcium, stabilizing the platelet plug by cross-linking individual fibrin molecules, which then form a stable fibrin network.17 The fibrin strands mesh with the platelets in the plug, making it insoluble.18

Subunit A is synthesized primarily by megakaryocytes, subunit B is synthesized in the liver.28

Factor XIII deficiency can occur from genetic defects (inherited factor XIII deficiency, from the production of antibodies that neutralize the mature factor XIII protein and inhibit the final steps of coagulation, or loss of factor XIII due to surgery or severe trauma.18

Factor XIII deficiency is very rare; the overall estimated frequency is one case per 2-5 million of the population. It can occur in either sex.18

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Last Updated: 4/23/2015 3:14 PM
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