Haemophilia A and B and von Willebrand disease account for 80-85% of all in
herited bleeding disorders. The other 15% are represented by deficiencies o
f fibrinogen, prothrombin, or factors V, VII, X, XI, or XIII. In addition,
acquired factor deficiencies are seen in a variety of conditions ranging fr
om malignancies to autoimmune disorders. The spectrum of symptoms in these
conditions varies from severe and life-threatening haemorrhage to a mild bl
eeding diathesis. The diagnosis depends on demonstration of decreased activ
ity of one of the clotting factors. Due to the rarity of each of the indivi
dual factor deficiencies, purified factor concentrates are not as readily a
vailable as they are for haemophilia A and B. Treatment of rare clotting fa
ctor deficiencies consists of the most purified blood product available tha
t contains the missing factor. Depending on which factor is deficient, eith
er purified concentrates, prothrombin complex concentrates, cryoprecipitate
, or fresh frozen plasma can be used. In addition, recombinant factor VIIa
is available for treating factor VII deficient patients.