Bleeding due to rare coagulation factor deficiencies

Rare coagulation factor disorders (RCD) are inherited conditions which put patients at risk of spontaneous and/or trauma-related bleeding. The patterns and the severity of bleeding depend on the underlying type of coagulation factor deficiency and, in some cases, the severity of the deficiency. Diagnosis can be challenging, particularly as many of these rare bleeding conditions are inherited in an autosomal recessive manner meaning that a child is often the first member of a family found to be affected. Rare bleeding conditions include deficiencies of fibrinogen as well as clotting factor deficiencies, i.e. factors II, V, VII, X, XI and XIII. Conditions such as haemophilia, von Willebrand disease and platelet defects are covered elsewhere. This chapter will provide an overview of the general management of inherited bleeding conditions as well as a short overview of each individual condition.