Congenital Factor XIII Deficiency – An Overview

Inherited Factor XIII deficiency is a rare bleeding disorder usually associated with severe bleeding from an early age. 2

A rare autosomal recessive disease

  • Incidence of FXIII deficiency is estimated at 1 in 3 million people.14
  • Affects all races and both sexes equally 3
  • Often runs in families 14

Caused by genetic defect in subunit of the Factor XIII molecule

  • Although abnormalities are commonly associated with mutations in the A-subunit, approximately 17% of patients have an abnormality in the B-subunit. 7
    • In patients with the B-subunit abnormality, survival of the FXIII molecule will be shorter. 7

A detectable deficiency

  • Clots form but break down within 24-48 hours. 2
  • Factor XIII deficiency may be missed by standard clotting tests, which detect the presence of a clot but not its stability; however, appropriate testing can lead to diagnosis. 3
  • A specific FXIII assay, which estimates FXIII activity, can then be used to confirm diagnosis. 3

Important Safety Information

CORIFACT®, FXIII Concentrate (Human), is indicated for routine prophylactic treatment and perioperative management of surgical bleeding in adult and pediatric patients with congenital Factor XIII deficiency. CORIFACT must be administered intravenously.

CORIFACT is contraindicated in individuals with known anaphylactic or severe systemic reactions to human plasma-derived products.

Hypersensitivity reactions may occur with CORIFACT. If there are signs of anaphylaxis or hypersensitivity reactions (including urticaria, rash, tightness of the chest, wheezing, and hypotension), immediately discontinue administration and institute appropriate treatment.

Inhibitory antibodies to FXIII have been detected in patients receiving CORIFACT. Monitor the patient’s trough FXIII activity level during treatment. If expected plasma FXIII activity levels are not attained or breakthrough bleeding occurs, perform an assay measuring FXIII inhibitory antibody concentrations.

Thromboembolic complications have been reported with CORIFACT; monitor patients with known risk factors for thrombotic events.

CORIFACT is derived from human blood. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent and its variant (vCJD), cannot be completely eliminated.

The most common adverse reactions reported in clinical trials (frequency >1%) following treatment with CORIFACT were joint inflammation, hypersensitivity, rash, pruritus; hematoma, arthralgia, headache, elevated thrombin-anti-thrombin levels, and increased blood lactate dehydrogenase. Serious adverse reactions included hypersensitivity, acute ischemia, and neutralizing antibodies against FXIII.

Please see full prescribing information for CORIFACT.

To report SUSPECTED ADVERSE REACTIONS, contact the CSL Behring Pharmacovigilance Department at 1-866-915-6958 or FDA at 1-800-FDA-1088 or