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Symptomatic individuals have <1% of normal FXIII6,18, resulting in unstable clot formations with reduced resistance to fibrinolysis18
The initial clot forms and bleeding stops, but bleeding can resume 24-48 hours later6
Wound healing problems have been described in 14% of patients4
It affects all races and genders equally, approximately one person in 1-2 million are affected worldwide7 8
Symptomatic individuals have <1% of normal FXIII6,18, resulting in unstable clot formations with reduced resistance to fibrinolysis18
The initial clot forms and bleeding stops, but bleeding can resume 24-48 hours later6
Wound healing problems have been described in 14% of patients4
It affects all races and genders equally, approximately one person in 1-2 million are affected worldwide7 8
1. Carcao M, Altisent C, Castaman G et al. Thromb Haemost 2018;118(3):451-460.
2. Carcao M, Fukutake K, Inbal A et al. Semin Thromb Hemost 2017;43(1):59-68.
3. Dorey E. Nat Biotechnol 2014;32:210.
4. Anwar R and Miloszewski KJ. Br J Haematol 1999;107(3):468-484.
5. Williams M, Will A, Stenmo C et al. Haemophilia 2014;20(1):99-105.
6. Hsieh L and Nugent D. Haemophilia 2008;14(6):1190-1200
7. Odame JE, Chan AK, Wu JK et al. Blood Coagul Fibrinolysis 2014;25(3):199-205.
8. Bolton-Maggs PHB. Treatment of Haemophilia. April 2006. No 39. World Federation of Hemophilia.
9. Inbal A, Oldenburg J, Carcao M et al. Blood 2012;119(22):5111-5117.
10. NovoThirteen® Summary of Product Characteristics.
11. Kerlin BA, Inbal A, Will A et al. J Thromb Haemost 2017;15(8):1601-1606.
12. Kerlin B, Brand B, Inbal A et al. J Thromb Haemost 2014;12(12):2038-2043.
13. Brand-Staufer B, Carcao M, Kerlin BA et al. Haemophilia 2015;21(3):380-385.
14. Fibrogammin® Summary of Product Characteristics.
15. Hvitfeldt Poulsen L, Kerlin BA, Castaman G et al. Res Pract Thromb Haemost 2020;4(Suppl 1). Abstract PB1169, ISTH 2020 Congress
16. European Medicines Agency. Guideline on plasma-derived medicinal products. Available at: https://www.ema. europa.eu/en/documents/scientific-guideline/guideline-plasma-derived-medicinal-products_en.pdf [Accessed December 2019].
17. Zanon E, Pasca S, Radu C et al. Haemophilia 2019;25(3):e206-e208.
18. Sharief LAT and Kadir RA. Haemophilia 2013;19(6):e349-e357.