Use of recombinant factor VIIa in hereditary bleeding disorders.

This is another excellent review of the mechanism of action, indications, and various doses of rFVIIa (Novoseven) in hereditary bleeding disorders. Its main use has been in patients with factor VIII or IX deficiency with inhibitors, where rFVIIa can by-pass factors VIII or IX, interacts with tissue factor and generates thrombin in addition to activating platelets. This has also lead to its efficacy in patients with inherited platelet dysfunction, such as Glanzmann thrombasthenia.

Several doses of rFVIIa have been used, as well as bolus versus continuous infusion. Early use achieves a better outcome. However, there is no available routine laboratory test which can be used to monitor the use of rFVIIa, and cost remains an important limiting factor. Adverse events are very small in number, especially the occurrence of thrombotic events or antibody formation.

– M. Aldouri.