Important

It is possible that the main title of the report Factor XIII Deficiency is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

• Fibrin Stabilizing Factor Deficiency
• Fibrinase Deficiency
• Fibrinoligase Deficiency
• Laki-Lorand Factor Deficiency
• Plasma Transglutaminase Deficiency

Disorder Subdivisions

• Congenital Factor XIII Deficiency
• Acquired Factor XIII Deficiency

General Discussion

Factor XIII Deficiency is an extremely rare inherited blood disorder characterized by abnormal blood clotting that may result in abnormal bleeding. Associated symptoms and findings occur as the result of a deficiency in the blood clotting factor F13A1 (Factor XIII). In affected individuals, the blood fails to clot appropriately, resulting in poor wound healing. Blood may seep into surrounding soft tissues, resulting in local pain and swelling. Internal bleeding may occur; approximately 25 percent of affected individuals experience bleeding in the brain (intracranial hemorrhage). Factor XIII Deficiency may be inherited as an autosomal dominant genetic trait. The disease may also be acquired in association with other disorders such as Sickle Cell Disease or Henoch-Schonlein Purpura.