Medication Summary

Scheduled factor XIII (FXIII) replacement every 4-6 weeks maintains factor XIII levels above the critical threshold for spontaneous bleeding and allows patients to participate in regular activities.

For patients with congenital factor XIII A-subunit deficiency, which includes the majority of patients, treatment with recombinant factor XIII, as well as treatment with factor XIII concentrate, can be considered.

For patients with a B-subunit deficiency, the recombinant product cannot be used.

For all patients, treatment with cryoprecipitate and fresh frozen plasma (FFP) should be considered in the event of acute bleeding if concentrate and recombinant factor are not available.

Class Summary

Hemostasis is the physiological response to bleeding. Injury to the blood vessel wall and factors released by platelets initiate the coagulation cascade. Formation of an insoluble fibrin clot, which reinforces the initial platelet plug, is mediated by blood clotting factors. Clotting factors function as cofactors in the blood-coagulation cascade.

Factor XIII A-subunit, recombinant (Tretten)

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Recombinant human factor XIII-A2 homodimer composed of 2 FXIII A-subunits. FXIII is the terminal enzyme in the blood coagulation cascade; when activated by thrombin at the site of vessel wall injury, FXIII plays an important role in the maintenance of hemostasis through cross-linking of fibrin and other proteins in the fibrin clot.

Factor XIII concentrate (Corifact)

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FXIII is a proenzyme that is activated, in the presence of calcium ion, by thrombin cleavage of the A-subunit to become activated FXIII (FXIIIa). Promotes cross-linking of fibrin during coagulation and is essential to the physiological protection of the clot against fibrinolysis.

Indicated for routine prophylactic treatment of congenital factor XIII (FXIII) deficiency.

Follow-up

Mangla A, Hamad H, Kumar A. Factor XIII Deficiency. StatPearls. 2021 Jan. [QxMD MEDLINE Link]. [Full Text].
Malkhassian D, Sabir S, Sharma S. Physiology, Factor XIII. StatPearls. 2021 Jan. [QxMD MEDLINE Link]. [Full Text].
Anwar R, Minford A, Gallivan L, Trinh CH, Markham AF. Delayed umbilical bleeding--a presenting feature for factor XIII deficiency: clinical features, genetics, and management. Pediatrics. 2002 Feb. 109(2):E32. [QxMD MEDLINE Link].
Karimi M, Peyvandi F, Naderi M, Shapiro A. Factor XIII deficiency diagnosis: challenges and tools. Int J Lab Hematol. 2018 Feb. 40 (1):3-11. [QxMD MEDLINE Link]. [Full Text].
Dickneite G, Herwald H, Korte W, Allanore Y, Denton CP, Matucci Cerinic M. Coagulation factor XIII: a multifunctional transglutaminase with clinical potential in a range of conditions. Thromb Haemost. 2015 Apr. 113 (4):686-97. [QxMD MEDLINE Link]. [Full Text].
Karimi M, Bereczky Z, Cohan N, Muszbek L. Factor XIII Deficiency. Semin Thromb Hemost. 2009 Jun. 35(4):426-38. [QxMD MEDLINE Link].
Naderi M, Alizadeh S, Kazemi A, et al. Central nervous system bleeding in pediatric patients with factor XIII deficiency: a study on 23 new cases. Hematology. 2015 Mar. 20 (2):112-8. [QxMD MEDLINE Link].
Gebhart J, Hofer S, Panzer S, et al. High proportion of patients with bleeding of unknown cause in persons with a mild-to-moderate bleeding tendency: results from the Vienna Bleeding Biobank (VIBB). Haemophilia. 2018 May. 24 (3):405-13. [QxMD MEDLINE Link].
Inbal A, Oldenburg J, Carcao M, Rosholm A, Tehranchi R, Nugent D. Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency. Blood. 2012 May 31. 119(22):5111-7. [QxMD MEDLINE Link].
Fadoo Z, Merchant Q, Rehman KA. New developments in the management of congenital Factor XIII deficiency. J Blood Med. 2013. 4:65-73. [QxMD MEDLINE Link]. [Full Text].
Rugeri L, Martinaud C, Beurrier P, et al. Gynecological and obstetric outcome in the French cohort of women with factor XIII deficiency. Thromb Res. 2020 Jul. 191:22-5. [QxMD MEDLINE Link]. [Full Text].
Naderi M, Eshghi P, Cohan N, Miri-Moghaddam E, Yaghmaee M, Karimi M. Successful delivery in patients with FXIII deficiency receiving prophylaxis: report of 17 cases in Iran. Haemophilia. 2012 Sep. 18(5):773-6. [QxMD MEDLINE Link].
Fahlbusch FB, Heinlein T, Rauh M, et al. Influence of factor XIII activity on post-operative transfusion in congenital cardiac surgery-A retrospective analysis. PLoS One. 2018. 13 (7):e0199240. [QxMD MEDLINE Link]. [Full Text].
Todd T, J Perry D. A review of long-term prophylaxis in the rare inherited coagulation factor deficiencies. Haemophilia. 2009 Nov 11. [QxMD MEDLINE Link].

Media Gallery

Activation of factor XIII (FXIII) by thrombin and calcium is a 2-step process. Thrombin cleaves an arginine-lysine bond in the A subunit and calcium causes dissociation of the B subunit, exposing the active site on the A subunit (XIIIa).

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