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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 XIIIfibrin
stabilization factoris 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.
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.
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 activityas 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.
With factor XIII deficiency, women have an increased risk of miscarriage.
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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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