A rare, genetic bleeding disorder characterized by deficiency of clotting factor XIII.
Patients form blood clots like normal but the clots are unstable and often break down, resulting in prolonged, uncontrolled bleeding episodes.
Factor XIII deficiency affects 1 in a million to 1 in five million people.
Signs include: Nosebleeds
Bleeding in the central nervous system
Bleeding in the mouth
Abnormal bleeding after injury or surgery
Bleeding from the umbilical cord
Diagnosis difficult as standard blood clotting tests does not detect the deficiency.
Specialized tests measure the amount of factor XIII in blood and can establish how factor XIII is working.
The high rate of bleeding at birth usually leads to early diagnosis.
Diagnosis includes: Patient history, clinical presentation, clot stability test, and quantitative assay.
Treatment is by Tretten a recombinant analogue of the human Factor XIII A-subunit.
Corifact (factor XIII concentrate) is indicated for routine prophylactic treatment and perioperative management of surgical bleeding in adult and pediatric patients with congenital Factor XIII deficiency
Cryoprecipitate is used only in threatening emergencies when FXIII concentrate is not available.