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)
Heterogeneity of type I von Willebrand disease : evidence for a subgroup with an abnormal von Willebrand factor / P.M. Mannucci, R. Lombardi, R. Bader, L. Vianello, A.B. Federici, S. Solinas, M.G. Mazzucconi, G. Mariani. - In: BLOOD. - ISSN 0006-4971. - 66:4(1985 Oct), pp. 796-802.
|Titolo:||Heterogeneity of type I von Willebrand disease : evidence for a subgroup with an abnormal von Willebrand factor|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
Settore MED/15 - Malattie del Sangue
|Data di pubblicazione:||ott-1985|
|Appare nelle tipologie:||01 - Articolo su periodico|