Use of human factor VIIa in the treatment of two hemophilia A patients with high-titer inhibitors.

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Abstract

Two patients with hemophilia A complicated with high-titer alloantibodies have been treated by repeated infusions of microgram quantities of pure human Factor VIIa. Patient 1 was presented with a gastrocnemius muscle bleeding that involved the knee joint. Upon treatment with Factor VIIa the circumference of the muscle decreased and joint mobility increased substantially. Patient 2 was given Factor VIIa concurrent with tranexamic acid in association with the extraction of two primary molars. No significant gingival bleeding occurred after Factor VIIa and tranexamic acid treatment. Furthermore, no deleterious side effects or increase of the alloantibody level were observed in either patient throughout the Factor VIIa infusion. These results, although limited and preliminary in nature, suggest that trace quantities of Factor VIIa can act as a Factor VIII bypassing activity and restore hemostasis in these patients.

Authors

U Hedner, W Kisiel