Consistency of responses to repeated DDAVP infusions in patients with von
Willebrand's disease and hemophilia A
F Rodeghiero, G Castaman, E Di Bona and M Ruggeri
Department of Hematology, San Bortolo Hospital, Vicenza, Italy.
The consistency in time of responses to separate desmopressin (DDAVP)
infusions in patients with von Willebrand's disease (vWD) and mild or
moderate hemophilia A has been the subject of limited investigation. We
report here the results of a clinical study undertaken to test the
consistency of responses to repeated DDAVP administrations in 22 patients
with vWD and 10 with mild or moderate hemophilia A (time interval between
first and last infusion ranging from 1 to 77 months; median, 13 months). In
patients with vWD, 80% of cases showed a departure of less than 20% from
the average VIII:C peak level calculated after the two infusions. A
similarly consistent pattern was observed for bleeding times recorded 30
minutes after each infusion. In patients with hemophilia A, some infused on
more than two instances, the departure from the average VIII:C peak level
was less than 20% in nearly 70% of cases. A good within-family consistency
was also demonstrated by analyzing data obtained from seven kindreds with
vWD and two with hemophilia A. In conclusion, our study suggests that the
pattern of responsiveness observed after a DDAVP test-infusion can be
reliably used to decide the future clinical management of the individual
patient and that a similar pattern of response is usually observed within
the same kindred.
Volume 74,
Issue 6,
pp. 1997-2000,
11/01/1989
Copyright © 1989 by The American Society of Hematology
