Questions? Contact us.

For drug information, click here.

Factor XIII deficiency is a rare blood disorder. People with the disorder have low levels of blood clotting factor XIII, leading to poor blood clotting, delayed wound healing, and abnormal bleeding. Factor XIII deficiency can be inherited or acquired. The inherited form affects 1–3 people per million people worldwide. Many never have significant enough symptoms to lead to a diagnosis.

People with factor XIII deficiency can bleed internally even after mild bumps or bruises. The blood can collect in surrounding soft tissues, and over time, it can form large cysts that cause nerve damage and destroy bone. Mild head trauma can lead to brain and spinal cord hemorrhage.

About 25 percent of people with factor XIII deficiency can experience brain and spinal cord hemorrhage after mild head trauma. Although the bleeding might stop on its own, it is still a serious risk factor for patients. Symptoms can include headaches, vomiting, seizures, and problems with speech, vision, and hearing.

Other symptoms can include blood in the urine, poor wound healing, abnormal wound bleeding, and blood blisters inside the abdomen. Pregnant women can miscarry if they don’t receive treatment. In men, factor XIII deficiency can cause low sperm counts or sterility. These problems often do not respond to treatment.

People with factor XIII deficiency should be careful using aspirin or other medications that can cause bleeding.

There are two types of factor XIII deficiency: inherited and acquired.

Inherited Factor XIII Deficiency
Inherited factor XIII deficiency stems from mutations of two genes that together control the body’s production of factor XIII. The mutations reduce the amount of functional factor XIII in the blood, potentially lowering it to less than 5 percent of normal levels.

Acquired Factor XIII Deficiency
Acquired factor XIII deficiency can be caused by several medical conditions, including hepatitis, cirrhosis, inflammatory bowel disease, overwhelming bacterial infections, several types of cancer, and abnormal immune system activation. The acquired form of the deficiency is relatively mild, with blood levels of factor XIII at 20–70 percent of normal. Patients usually don’t have bleeding episodes if levels are above 10 percent of normal.

Inherited factor XIII deficiency can be treated with Corifact® (factor XIII concentrate [human]), generally infused every 3-4 weeks for prophylactic dosing. Alternatively, patients with factor XIII A subunit deficiency can also be treated with Tretten® (coagulation factor XIII A-subunit [recombinant]). Because the blood only needs about 10 percent of the standard level of factor XIII to respond normally to trauma, the doses can be small. Patients can also receive transfusions of cryoprecipitates (blood plasma), but this is an emergency measure.

Corifact and Tretten are available through Diplomat Specialty Infusion Group. Patients on these medications must be careful not to exceed the labeled dose, as high doses can lead to abnormal clotting and side effects.

Sources: Genetic and Rare Diseases Information Center, Genetics Home Reference, MedScape

Reference to a specific product, process, or service does not constitute an endorsement or recommendation by Diplomat Pharmacy Inc., and any such reference is provided for educational purposes only.

Products We Support


  • STABILIZE logo

    STABILIZE helps factor XIII deficiency patients by providing personal care representatives, home-delivery of factor, education, reimbursement coordination and more.

  • REBUILD logo

    Physical therapy is key to comprehensive hemophilia management. REBUILD facilitates training and connections between hemophilia patients and their local physical therapists.

  • IMPACT logo

    A comprehensive in-home inhibitor management program, IMPACT helps families affected by hemophilia with inhibitor options, education and personal support.


  • Can I Use Diplomat?

    Can I Use Diplomat?

    We provide benefits investigation and help verify your eligibility for our programs and services. Fill out a simple form and we’ll get in touch with you.
  • Financial Assistance

    Financial Assistance

    We help connect patients with nonprofit, manufacturer-sponsored and third-party funding options to keep therapy affordable.
  • Hemophilia of North Carolina Educational Scholarship Program

    Hemophilia of North Carolina Educational Scholarship Program

    All bleeding disorder patients, siblings, caregivers or parents pursuing higher education or professional training are encouraged to apply.
  • It’s Always About Nate

    It’s Always About Nate

    The fourth in the “Nate” series, this children’s story explores how bleeding disorders affect parents’ and siblings’ lives too—and how a little compassion can go a long way.
  • The Great Inhibinator

    The Great Inhibinator

    By working through his feelings with his family, six-year-old Nate finds a way to feel powerful despite his inhibitor diagnoses.
  • StrapWrap®


    Able to fasten to seatbelts, bag straps and bicycles, the StrapWrapⓇ keeps medical history and information secure and accessible to emergency responders.
  • Hemophilia Newsletter

    Hemophilia Newsletter

    Stay current on news, tips, stories, advancements and more from the hemophilia community. Available via email or print.
  • Kids Hemophilia Calendar Contest

    Kids Hemophilia Calendar Contest

    Children with bleeding disorders and their families are invited to submit artwork for this year’s calendar contest.
  • Be a Hero Mat

    Be a Hero Mat

    This mat provides a clean surface to help children learn to infuse at home, with clear instructions and colorful illustrations.

Featured Technology

A mobile health care application for patients with hemophilia and related bleeding disorders. The app helps patients conveniently track their personal infusion records and bleeding event history, as well as share important injury and treatment information with members of their health care team.

Learn more

Receive more information on what matters to you.