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Blood, 1 January 2002, Vol. 99, No. 1, pp. 180-184
HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
Reduced von Willebrand factor survival in type Vicenza von
Willebrand disease
Alessandra Casonato,
Elena Pontara,
Francesca Sartorello,
Maria Grazia Cattini,
Maria Teresa Sartori,
Roberto Padrini, and
Antonio Girolami
From the Department of Medical and Surgical Sciences,
Second Chair of Internal Medicine, and the Department of Pharmacology
and Anesthesiology, University of Padua Medical School, Italy.
Type Vicenza variant of von Willebrand disease (VWD) is
characterized by a low plasma von Willebrand factor (VWF) level and supranormal VWF multimers. Two candidate mutations, G2470A and G3864A
at exons 17 and 27, respectively, of the VWF gene were recently reported to be present in this disorder. Four additional families, originating from northeast Italy, with both mutations of type
Vicenza VWD are now described. Like the original type Vicenza
subjects, they showed a mild bleeding tendency and a significant decrease in plasma VWF antigen level and ristocetin cofactor activity but normal platelet VWF content. Unlike the original patients, ristocetin-induced platelet aggregation was found to be normal. Larger
than normal VWF multimers were also demonstrated in the plasma.
Desmopressin (DDAVP) administration increased factor VIII (FVIII) and
VWF plasma levels, with the appearance of even larger multimers.
However, these forms, and all VWF oligomers, disappeared rapidly from
the circulation. The half-life of VWF antigen release and of
elimination was significantly shorter than that in healthy counterparts, so that at 4 hours after DDAVP administration, VWF antigen levels were close to baseline. Similar behavior was
demonstrated by VWF ristocetin cofactor activity and FVIII. According
to these findings, it is presumed that the low plasma VWF levels of
type Vicenza VWD are mainly attributed to reduced survival of the VWF molecule, which, on the other hand, is normally synthesized. In addition, because normal VWF-platelet GPIb interaction was observed before or after DDAVP administration, it is proposed that type Vicenza
VWD not be considered a 2M subtype.
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