Pathophysiology

Deficiency of other coagulation factors is described but with a prevalence of 1-2 per million is rare cf. vWD and haemophilia A and B. Autosomal recessive inheritance, the deficiency either due to reduced synthesis (type 1) or production of a variant protein (type 2). All coagulation factors are produced in the liver and their interaction in the coagulation cascade is shown.

• The ti/2 of the factors vary and will determine the frequency and ease of treatment.

• Factor concentrates should be considered when available, e.g. factor XI, factor XIII.

• Recombinant VIIa for factor VII deficiency. The use of recombinant factor VIIa is increasing and it is increasingly used to treat a variety of factor deficiencies and severe platelet function disorders.

• FFP can be used and virally-inactivated plasma should be used when available. FFP is a source of all coagulation factors but large volumes may be required and even with viral inactivation there is risk of disease transmission.

0 0

Post a comment