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. 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.