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HEMOPHILIA.ORG > BLEEDING DISORDERS INFO CENTER > TYPES >
FACTOR XIII DEFICIENCY


Factor XIII Deficiency
(Fibrin Stabilizing Factor)


What Is It?

This is perhaps the rarest of all factor deficiencies, affecting just one in several million. A hallmark of this rare inherited deficiency is poor wound healing and abnormal scar formation. The reason is that factor XIII—fibrin stabilization factor—is necessary for clot formation and wound healing.
Factor XIII is responsible for clot stabilization and cross linking of the fibrin polymer in blood. A clot will form in the absence of factor XIII, but it will be unstable.

Factor XIII deficiency is a severe bleeding disorder usually associated with trauma. Typically, it's discovered in newly-born babies who suffer recurrent bleeding from the umbilical stump and hemorrhages after circumcision.

Severely affected patients have a high incidence of intracranial hemorrhage with little or no trauma. Affected women often experience spontaneous abortion unless treated with plasma replacement therapy. Bleeding in surgery is not excessive, but delayed bleeding can occur.

Inheritance Pattern
The disorder is not sex-linked as is hemophilia. It affects both males and females with equal frequency. It is an autosomal recessive disorder, which means if the clotting defect is inherited from a parent, the child will be a genetic carrier of the condition, but may or may not have symptoms.

Symptoms & Diagnosis
The most common clinical symptom, seen in over 80% of cases, is bleeding from umbilical stump after birth and prolonged bleeding from trauma. Bleeding episodes are usually lifelong and include severe bruising, hematomas, and prolonged bleeding after trauma.

Characteristically, bleeding is delayed for several hours or days after trauma. Hemorrhage into the brain or spinal cord area is more common than in other inherited coagulation disorders and can be life-threatening. Plasma replacement is given to pregnant women to prevent spontaneous abortions.

Without replacement therapy, patients in later life frequently sustain superficial bruising, epistaxes, hematoma in muscles and joints, and intracranial hemorrhage.

Specific factor XIII assays are needed to confirm a diagnosis of this deficiency. Usual tests of coagulation give normal results.

Treatments
Deficiency of factor XIII can be corrected with infusions of fresh frozen plasma, cryoprecipitate, or factor XIII concentrates. Because of the high incidence of intracranial bleeding and spontaneous abortions, prophylaxis is often recommended. Minimal factor XIII activity—as little as 5%—is required to prevent bleeding complications.

A plasma-derived, pasteurized factor VIII concentrate is produced by Behringwerke in Germany, but is not licensed for use in the U.S. The half-life of factor XIII is around 10 days.

The National Hemophilia Foundation's Medical and Scientific Advisory Council (MASAC) made recommendations for treatment of factor XIII deficiency in November of 1999. They include:
• Fibrogammin P, a plasma-derived factor XIII concentrate, though it is not yet licensed in the U.S.

Complications
With factor XIII deficiency, women have an increased risk of miscarriage.

Disclaimer
The information contained on the NHF web site is provided for your general information only. NHF does not give medical advice or engage in the practice of medicine. NHF under no circumstances recommends particular treatment for specific individuals and in all cases recommends that you consult your physician or local treatment center before pursuing any course of treatment.
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