What is the role of disorders of fibrin stabilization in the etiology of factor XIII (FXIII) deficiency?

Updated: Aug 01, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

Disorders of fibrin stabilization can affect the activity of FXIII or its substrates fibrin and fibrinogen. A proposed classification of disorders leading to a positive urea solubility test result is presented below.

Abnormalities of FXIII (enzyme) are as follows:

  • Genetic mutation - (1) Subunit A, (2) subunit B, (3) subunit A and B

  • Acquired - (1) Decreased production (ie, liver disease), (2) increased loss due to excessive activation (ie, DIC, exposure to snake venoms and caterpillar toxins), (3) secondary to inhibitors (ie, alloantibodies and autoantibodies)

Abnormalities of the substrate for FXIII (fibrin/fibrinogen) are as follows:

  • Genetic mutation - (1) Afibrinogenemia, (2) dyshypofibrinogenemia

  • Acquired - (1) Decreased production (ie, acute massive hepatic necrosis or severe chronic liver disease), (2) increased loss resulting from defibrination syndromes (ie, DIC, exposure to snake venoms and caterpillar toxins, and systemic hyperfibrinolysis [drug induced or disease induced])


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