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.
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.
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.
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.
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