ABSTRACT
-Patients with hemophilia may develop alloantibodies (inhibitors) directed toward the substitution factor. In the long term, this complication significantly affects around 15% of hemophilia A patients and 2-5% of hemophilia B patients suffering the severe class of disease (critical functional factor level <0.01 IU/mL). The typical consequence of a newly developed inhibitor is that the patient can no longer take advantage of the principles of modern hemophilia management, including safe and early treatment of bleeding and prophylactic use of concentrate in prevention of bleeding. Moreover, problems that are normally solved by means of a surgical procedure covered by the usual concentrate will be regarded as difficult and are likely to be associated with risk of untoward bleeding if high-titer inhibitors are present. Over the past decade, considerable experience has been collected illustrating that surgery may be performed quite safely in the patient with inhibitors if hemostasis is assisted by a new hemostatic agent, an activated recombinant factor VII molecule (rFVIIa). Although only one controlled randomized surgical study has been presented, cumulated data from this and from other, less formal studies on the use of rFVIIa in hemophilic inhibitor surgery have illustrated that a vast variety of different surgical procedures can be accomplished with none of the life-threatening bleeding complications that would be anticipated if no hemostatic treatment was administered. The aim of the present review is to present a multiplicity of surgical procedures that have been carried out during the clinical development of rFVIIa. Further, an update of 21 surgical procedures in inhibitor patients from the center of the author will be reviewed and discussed.
KEYWORD
Hemophilia - bypassing agent - inhibitors - FVIIa - recombinant factor VIIa - surgery
