A Review of Long-term Prophylaxis in the Rare Inherited Coagulation Factor Deficiencies

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.