What is factor XIII deficiency?

Updated: Oct 28, 2019
  • Author: Muhammad A Mir, MD, FACP; Chief Editor: Perumal Thiagarajan, MD  more...
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Factor XIII deficiency is a decrease or absence of factor XIII (fibrin-stabilizing factor [FSF]) that prevents blood-clot formation and results in a clinical hemorrhagic diathesis. Factor XIII is an enzyme found in plasma, platelets, and monocytes. In plasma, factor XIII has 2 subunits: the a subunit, which is the active enzyme and the b subunit, which is a carrier protein. [113] Activated factor XIII stimulates cross-linkage of fibrin as a means of stabilizing clot.

A bleeding patient with both normal PT and aPTT should raise the suspicion.

Congenital factor XIII deficiency is a severe autosomal recessive bleeding disorder associated with a characteristic pattern of neonatal hemorrhage and lifelong bleeding diathesis. Untreated patients have a high mortality rate. Even relatively minor trauma can be followed by prolonged and recurrent bleeding. Intracranial hemorrhage is a frequent complication. [114] The disorder affects both sexes, and bleeding may occur during pregnancy. [115] Acquired factor XIII deficiency has been described in HSP, various forms of colitis, erosive gastritis, and some forms of leukemia. Inhibitors to factor XIII are rare. [116]

Treatment of factor XIII deficiency requires lifelong prophylactic therapy with at least monthly infusions of factor XIII concentrate, even during pregnancy. [114, 115] . A recombinant factor XIII A-subunit (Tretten) is approved for routine prophylaxis of bleeding in patients with congenital factor XIII. [117]

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