887 INTRANASAL DDAVP IN PATIENTS WITH VON WILLEBRAND'S DISEASE AND HEMOPHILIA A

Abstract

The effect of a single intranasal dose (200 ugm) of 1-deamino-9-D-Arginine Vasopressin (DDAVP) was studied in 12 individuals with von Willebrand's disease (vWD) and 2 with moderate hemophilia A. Crossed immunoelectrophoresis of VIII:RAg demonstrated normal electrophoretic mobility in each of the vWD subjects. Components of the factor VIII system (VIII:C, VIII:RAg,VIII:R Cof) were assayed pre- and 90 and 180 minutes post-DDAVP. Each of 11 subjects with mild or moderate vWD had an increase in VIII:C activity (avg. 2X increase), 8 of 11 had an increase in VIII R: Cof, and 9 of 11 had an increase in VIII:RAg. The twelfth vWD subject, who had severe vWD, had no rise in any of these components. Of 4 vWD subjects who had pre- and post-DDAVP template bleeding times (BT) performed, the only one who had a prolonged baseline BT showed a normal BT 90 minutes post-DDAVP. One vWD subject who had demonstrated an increase in all F VIII components after DDAVP, later underwent dental extractions 90 minutes after DDAVP. No excessive bleeding was noted. Two individuals with moderate hemophilia A (baseline VIII:C values of 0.02 u/ml and 0.07 u/ml) were also studied. One had a rise in all components of the factor VIII system post-DDAVP while the other did not. We conclude that DDAVP results in transient improvement in selected individuals with vWD or moderate hemophilia A. This drug thus warrants further study as an alternative to blood components in the management of vWD and mild hemophilia.

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