About Factor XIII Deficiency

What is Factor XIII Deficiency?

Factor XIII Deficiency is a rare inherited disorder in which the body does not produce enough of a protein called Factor XIII. This protein is important for the normal clotting of blood. People with this disorder may experience excessive bleeding after an injury or surgery, and may be at risk for life-threatening bleeding in the brain or other organs. Treatment typically involves regular infusions of Factor XIII concentrate.

What are the symptoms of Factor XIII Deficiency?

The symptoms of Factor XIII Deficiency can vary from person to person, but may include:

- Prolonged bleeding after surgery, injury, or childbirth
- Easy bruising
- Excessive bleeding from the gums or nose
- Heavy menstrual bleeding
- Blood in the urine or stool
- Delayed healing of cuts or wounds
- Anemia due to chronic blood loss
- Swelling and Pain in joints due to bleeding into the joint space

What are the causes of Factor XIII Deficiency?

Factor XIII Deficiency is a rare inherited disorder caused by mutations in the F13A1 gene. This gene provides instructions for making an enzyme called factor XIII A, which is involved in the clotting process. Mutations in this gene can lead to a deficiency of factor XIII A, which can cause excessive bleeding. Other causes of Factor XIII Deficiency include acquired conditions such as liver disease, kidney failure, and certain types of cancer.

What are the treatments for Factor XIII Deficiency?

The main treatment for Factor XIII Deficiency is regular infusions of Factor XIII concentrate. This is a protein that helps the blood to clot and is given intravenously. Other treatments may include medications to reduce the risk of bleeding, such as antifibrinolytics, and blood transfusions. In some cases, surgery may be necessary to stop bleeding.

What are the risk factors for Factor XIII Deficiency?

1. Family history of Factor XIII Deficiency
2. Certain ethnic backgrounds, including Ashkenazi Jews, French Canadians, and Finns
3. Certain genetic mutations, including those in the F13A1 gene
4. Pregnancy
5. Liver disease
6. Certain medications, such as anticonvulsants and antibiotics
7. Radiation therapy
8. Certain medical conditions, such as cancer, HIV/AIDS, and autoimmune disorders

Is there a cure/medications for Factor XIII Deficiency?

Yes, there is a treatment for Factor XIII Deficiency. The treatment involves regular infusions of a clotting factor concentrate, which is a medication that helps to replace the missing clotting factor. This treatment helps to reduce the risk of bleeding episodes and can help to improve the quality of life for those with the condition.