What is factor XIII (FXIII) deficiency?

Updated: Aug 01, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Factor XIII (FXIII), which was initially termed fibrin stabilizing factor, is involved in clot preservation. FXIII also participates in other physiologic processes, including wound repair and healing. FXIII deficiency, an autosomal recessive disorder, is a rare but potentially life-threatening cause of a hemorrhagic diathesis. Paradoxically, alterations in FXIII may also predispose to thrombosis. 

Congenital FXIII deficiency is due principally to defects in the catalytic A subunit of FXIII, with more than 100 mutations throughout the factor XIII A gene identified. [1]  Acquired FXIII deficiencies, which result from autoantibodies against FXIII subunits, are extremely rare but may produce severe bleeding diatheses. [2]

Thrombin, generated by reactions initiated by activated tissue factor VII/factor IX pathways, leads to clot formation. See the image below.

Final steps in clot formation (from article: Facto Final steps in clot formation (from article: Factor XIII).

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