Skip to main content

What is Factor XIII Deficiency?

Factor XIII deficiency is a rare inherited bleeding disorder in which Factor XIII, the protein in the blood responsible to help stabilize a blood clot, is malfunctioning or deficient. If a person is deficient in Factor XIII, the body is able to form blood clots, but they often are weak and therefore break down, resulting in prolonged bleeding.

According to the National Organization of Rare Disorders (NORD), the incidence of Factor XIII deficiency has been estimated to be between 1 in 2-5 million people in the general population.

Most people with Factor XIII deficiency typically have normal lifespans. However, it’s important for patients to work with their doctors to effectively monitor their condition to avoid potential complications. For example, since Factor XIII deficiency can cause issues with bleeding after surgery, it’s important that doctors are aware ahead of time so that they can plan the procedure accordingly. Factor XIII deficiency also involves an increased risk of miscarriage, but working with medical professionals can help reduce that risk in pregnant people.

Symptoms of Factor XIII Deficiency

Symptoms of Factor XIII deficiency commonly begin to show shortly after birth with umbilical cord bleeding. Spontaneous intracranial hemorrhage is a common occurrence with factor XIII deficiency, occurring in approximately 30% of people. Because factor XIII deficiency affects blood clotting, people may also experience bleeding complications after surgery or after injury.

Other symptoms of factor XIII deficiency include:

  • Soft tissue bleeds
  • Nose and mouth bleeds
  • Bleeding in joints
  • Menorrhagia (heavy menstrual bleeding)
  • Easy bruising

Screening for Factor XIII Deficiency

If there is a family history of Factor XIII deficiency, the deficiency can be screened for during pregnancy through tests such as amniocentesis or chorionic villus sampling. After a person is born, a test can be done to check the stability of blood clots once they form.

Factor XIII Deficiency Resources for You