Factor XIII Deficiency

What is factor XIII deficiency?

Factor XIII deficiency, also known as fibrin stabilizing deficiency, is a rare genetic bleeding disorder. Factor XIII deficiency leads to abnormal and prolonged bleeding due to ineffective clot formation. Individuals with this disorder form clots, but they are unstable and fail to work. Factor XIII deficiency leads to poor wound healing and problems with pregnancy.  It is estimated that factor XIII deficiency occurs in 1 in 5 million births.

What are the symptoms of factor XIII deficiency?

The symptoms of factor XIII deficiency are related to ineffective clotting of the blood. The symptoms vary in severity from individual to individual and include:

• Prolonged umbilical cord bleeding at birth
• Bleeding in brain or spinal cord, even after a mild trauma
• High miscarriage risk in pregnant females
• Heavy and prolonged menstrual bleeding
• Low sperm counts or sterility in males
• Poor wound healing
• Blood in urine
• Blood in joints
• Internal bleeding

What causes factor XIII deficiency?

Factor XIII deficiency is an inherited blood disorder and must be passed from both the mother and the father.  This disorder is also thought to be acquired from liver failure, inflammatory bowel disorder, and myeloid leukemia.

Are there treatment options for factor XIII deficiency?

Factor XIII deficiency is treated with infusions of fresh and frozen blood plasma or factor XIII concentrate.  These infusions can lead to a normal response to trauma for a patient.  If symptoms are severe or persistent such as with brain bleeds, preventative infusions are done. A drug known as Corifact was approved by the FDA in 2011 for treatment of factor XIII deficiency and the prevention of symptoms and complications.

Where can I find more information about factor XIII deficiency?

• NIH
• Hemophilia