The use of long-term prophylactic factor replacement therapy with rVIIa 15-30 µg/kg 2-3 times per week has been described to be effective in patients with a severe deficiency of FVII and with a bleeding phenotype [1]. Similar results were shown in the STER-registry using a total weekly dose of 90 µg/kg/week [2] and also in children [3].
0.1 Recommendation for long-term prophylactic factor replacement therapy
Long-term prophylactic factor replacement therapy should be considered in severe FVII deficiency with severe bleeding phenotype.
rFVIIa initially 15-30 µg/kg 2 -3 times weekly followed by individualized dose tailoring is suggested.
1.
Tengborn L, Blombäck M, Berntorp E. Tranexamic acid – an old drug still going strong and making a revival. Thrombosis Research 2015;135:231–42. doi:10.1016/j.thromres.2014.11.012.
2.
Napolitano M, Giansily-Blaizot M, Dolce A, Schved JF, Auerswald G, Ingerslev J, et al. Prophylaxis in congenital factor VII deficiency: indications, efficacy and safety. Results from the Seven Treatment Evaluation Registry (STER). Haematologica 2013;98:538–44. doi:10.3324/haematol.2012.074039.
3.
Kuperman A, Barg A, Fruchtman Y, Shaoul E, Rosenberg N, Kenet G, et al. Primary prophylaxis for children with severe congenital factor VII deficiency — Clinical and laboratory assessment. Blood Cells, Molecules, and Diseases 2017;67:86–90. doi:10.1016/j.bcmd.2016.12.008.