What is it?
(Can also be known as Fibrin Stabilizing Factor deficiency)
This condition is perhaps the rarest of all factor deficiencies. The incidence of Factor XIII deficiency is estimated at one in five million births. It is inherited in an autosomal recessive fashion, which means it affects men and women equally. No racial or ethnic group is disproportionately affected.
Factor XIII is the protein responsible for stabilizing the formation of a blood clot. In the absence of Factor XIII, a clot will still develop but it will remain unstable. When someone has a deficiency of Factor XIII, the tenuously formed clot will eventually break down and cause recurrent bleeds. The prolonged bleeding that is associated with Factor XIII is usually associated with trauma. Among severe patients there is a high risk of head bleeds with or without trauma. Bleeding immediately after surgery is usually not excessive, but can be delayed. Women who go untreated risk spontaneous abortion. Men with the deficiency may show signs of infertility. Common characteristics include soft tissue bleeds, menorrhagia, joint bleeding, and persistent bleeding during circumsicion or at the site of the umbilical cord.
