A review of long‐term prophylaxis in the rare inherited coagulation factor deficiencies

Summary. The rare inherited coagulation factor deficiencies (deficiencies of factors I, II, V, VII, XI, XIII, combined FV + FVII deficiency, combined deficiency of the vitamin K dependent factors and von Willebrand disease type 3) have an aggregate prevalence of approximately 1:100 000. They may cause recurrent life or function threatening haemorrhage. In this article we review the available literature on long‐term prophylaxis and, where possible, make recommendations on this important area.