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Heterogeneity of type I von Willebrand disease: evidence for a subgroup
with an abnormal von Willebrand factor
PM Mannucci, R Lombardi, R Bader, L Vianello, AB Federici, S Solinas, MG Mazzucconi and G Mariani
Type I von Willebrand disease (vWD) is characterized by equally low plasma
concentrations of von Willebrand factor antigen (vWF:Ag) and ristocetin
cofactor (RiCof) and by the presence of all vWF multimers in sodium dodecyl
sulfate (SDS)-agarose gel electrophoresis. For 17 patients (13 kindreds)
diagnosed with these criteria, we have studied the platelet contents of
vWF:Ag and RiCof and the changes of these in plasma after DDAVP infusion.
Platelet vWF:Ag and RiCof were normal in four kindreds (called "platelet
normal" subgroup); following 1-deamino- 8-D-arginine vasopressin; plasma
vWF:Ag, RiCof and the bleeding time (BT) became normal. In six kindreds,
platelet vWF:Ag and RiCof were equally low (platelet low); after DDAVP,
plasma vWF:Ag and RiCof remained low, and the BT was prolonged. In three
additional kindreds, platelets contained normal concentrations of vWF:Ag,
but RiCof was very low (platelet discordant); even though a complete set of
multimers was found in plasma and platelets, there was a relatively small
amount of large multimers. After DDAVP, plasma vWF:Ag became normal, but
RiCof remained low and the BT was very prolonged. These findings
demonstrated that there can be an abnormal vWF (RiCof less than vWF:Ag)
even in type I vWD, coexisting with a complete set of vWF multimers
(platelet discordant); that the abnormal vWF can be shown more clearly in
platelets than in plasma or else in plasma after DDAVP infusion; and that
DDAVP normalizes the BT only in those patients with normal platelet levels
of both vWF:Ag and RiCof (platelet normal).
Volume 66,
Issue 4,
pp. 796-802,
10/01/1985
Copyright © 1985 by The American Society of Hematology
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