DDAVP: a useful alternative to blood components in moderate hemophilia A and von Willebrand disease.

The effect of the vasopressin analog 1-deamino-8-d-arginine vasopressin (DDAVP) was studied in three normal individuals, 31 subjects with von Willebrand disease, and seven subjects with mild or moderate hemophilia A. None of those with von Willebrand disease had qualitative abnormalities of factor VIII-related antigen (F VIII:RAg). Both intranasal (2 to 4 micrograms/kg) and intravenous (0.2 micrograms/kg) DDAVP were used. All normal subjects, 27 of 31 with von Willebrand disease, and six of seven with hemophilia had a more than 200% increase in F VIII coagulant activity, with lesser but definite increases in F VIII:RAg and ristocetin cofactor activity. Two subjects with severe von Willebrand disease had no increase in F VIII-related activities. In six subjects with von Willebrand disease who had prolonged bleeding times, there was transient correction after DDAVP therapy. None of eight subjects who received DDAVP prior to surgical procedures had any unusual bleeding during or after surgery. None received any blood products. No untoward side effects were noted in any of the 41 subjects. We conclude that DDAVP is a safe and effective alternative to the use of blood products in certain individuals with von Willebrand disease and hemophilia A.