Factor Fibrinogen (Factor XIII) Deficiency
Understanding Factor XIII Deficiency
Inherited factor XIII deficiency is the rarest factor deficiency, occurring in 1 out of 5 million births. It is inherited from both parents and affects men and women equally. Acquired forms of factor XIII deficiency have been reported in association with liver failure, inflammatory bowel disease, and myeloid leukemia.
Symptoms of Factor XIII Deficiency
Symptoms of factor XIII deficiency include bruise easily, recurrent bleeds including nose and mouth, muscle bleeds, and delayed bleeding after surgery. Umbilical cord bleeding is common in factor XIII deficiency, found in almost 80% of cases. Up to 30% of patients sustain a spontaneous intracranial hemorrhage, a brain bleed. This is the leading cause of mortality for those with factor XIII deficiency. Females with factor XIII deficiency who become pregnant have a high risk for miscarriage without appropriate treatment.
Diagnosing Factor XIII Deficiency
Diagnosing factor XIII deficiency is made using factor XIII assays and a clot solubility test.
Treating Factor XIII Deficiency
Treatment available for factor XIII deficiency includes factor concentrate, cryoprecipitate and fresh frozen plasma.
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