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Studies of the pathophysiology of acquired von Willebrand's disease in
seven patients with lymphoproliferative disorders or benign monoclonal
gammopathies
PM Mannucci, R Lombardi, R Bader, MH Horellou, G Finazzi, C Besana, J Conard and M Samama
In seven patients with acquired von Willebrand's disease (AvWD) associated
with lymphoproliferative disorders or benign monoclonal gammopathies, the
platelet contents of von Willebrand factor antigen and ristocetin cofactor
(vWF:Ag and vWF:RiCof, respectively) were normal. All the multimers of
vWF:Ag could be seen in the 1.6% SDS- agarose gel electrophoresis patterns
of plasma and platelet lysates. Infusion of 1-deamino-8-D-arginine
vasopressin (DDAVP) augmented plasma levels of vWF:Ag and vWF:RiCof of all
patients and corrected prolonged bleeding times (BT). However, compared
with patients with congenital vWD type I and comparable degrees of baseline
abnormalities treated in the same way, vWF:Ag and vWF:RiCof were increased
less and cleared more rapidly from plasma and the BT remained normal for a
shorter period of time. These studies provide evidence that these AvWD
patients have qualitatively normal vWF in plasma, but at lower
concentrations, that vWF in platelets is normal both qualitatively and
quantitatively, and that cellular vWF can be rapidly released into plasma
by DDAVP to correct the hemostatic abnormalities. However, vWF is removed
rapidly from plasma, making the correction more transient than in
congenital vWD type I.
Volume 64,
Issue 3,
pp. 614-621,
09/01/1984
Copyright © 1984 by The American Society of Hematology
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