After von Willebrand disease, this is the most common bleeding disorder affecting females. The incidence of factor XI deficiency is 1 in 100,000, though it occurs more frequently among members of some ethnic groups. Its incidence is about 1 in 10,000 among Ashkenazi Jews. There are at least three different known genetic changes associated with factor XI deficiency; these vary in their effect on bleeding. Your doctor can explain exactly what type of factor XI deficiency you have and how this will affect your care and treatment.

Factor XI deficiency is usually diagnosed after injury-related bleeding, and symptoms tends to be mild. Nearly 50% of people with factor XI deficiency report no bleeding problems.

Individuals with factor XI deficiency may experience bruising, nosebleeds, or blood in their urine. Some women have prolonged bleeding after childbirth. Spontaneous bleeding is uncommon, even in people with severe factor XI deficiency. Joint bleeding is uncommon, but delayed bleeding (bleeding long after an injury has occurred) can be a problem.

Treatment Options
No commercial concentrate of factor XI is available in the United States. Accordingly, the treatment of choice for factor XI deficiency is plasma.