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Factor XIII deficiency is an extremely rare inherited blood disorder characterized by abnormal blood clotting that may result in abnormal bleeding. Signs and symptoms occur as the result of a deficiency in the blood clotting factor 13, which is responsible for stabilizing the formation of a blood clot. 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; about 25 percent of affected individuals experience bleeding in the brain. FXIII deficiency is usually caused by mutations in the F13A1 gene, but mutations have also been found in the F13B gene. It is usually inherited in an autosomal recessive fashion. Acquired forms have also been reported in association with liver failure, inflammatory bowel disease, and myeloid leukemia.
Source: GARD Last updated on 05-01-20
Factor XIII replacement is used to treat bleeding, to prevent bleeding during surgical procedures, or to prevent recurrent bleeding (such as central nervous system or joint hemorrhages). More detailed information about the treatment of factor XIII deficiency is available on the Medscape Reference website. You may need to register to view the article, but registration is free.
Last updated on 05-01-20
Read more about Factor XIII deficiency on the National Hemophilia Foundation Web site.
Last updated on 04-27-20
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