Journal of Clinical Oncology, Vol 10, 422-427, Copyright © 1992 by American Society of Clinical Oncology
Acquired von Willebrand disease in Wilms' tumor patients
MJ Coppes, SW Zandvoort, CR Sparling, AO Poon, S Weitzman and VS Blanchette
Department of Paediatrics, Hospital for Sick Children, Toronto, Canada.
PURPOSE: A prospective study was performed to determine the incidence of
acquired von Willebrand disease (vWD) in children with newly diagnosed
Wilms' tumor. PATIENTS AND METHODS: Fifty consecutive children with newly
diagnosed Wilms' tumor were evaluated. Detailed family and bleeding
histories were obtained in all cases. Laboratory evaluation included
measurement of the circulating platelet count, bleeding time (BT), factor
VIII (FVIII) and von Willebrand factor (vWF) levels, and ristocetin
cofactor (RCoF) activity. A vWF multimer analysis was obtained in all cases
in which vWD was suspected. RESULTS: Four of 50 (8%) consecutive children
with a diagnosis of Wilms' tumor were found to have acquired vWD.
Laboratory findings indicated type III vWD in two patients and type I vWD
in the other two. CONCLUSIONS: The incidence of acquired vWD in association
with Wilms' tumor merits further study through a large prospective trial.
Such a trial should include careful family and clinical bleeding histories
plus measurement of a platelet count, BT, coagulant FVIII and vWF levels,
RCoF activity, and vWF multimer analysis. The response to
1-desamino-8-D-arginine vasopressin (DDAVP) should be tested in all
patients with Wilms' tumor and acquired vWD, including patients with a type
III profile, before an invasive procedure is performed. Successful use of
DDAVP may avoid exposure of affected patients to blood products.
