Int J Lab Hematol. 2008 Apr;30(2):91-4.

Clinical, laboratory and therapeutic aspects of platelet-type von Willebrand disease.

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Servizio di Immunoematologia e Trasfusione, Centro Emofilia, Azienda Ospedaliera di Verona, Verona, Italy. mfranchini@univr.it

Abstract

Platelet-type von Willebrand disease (PT-VWD), or pseudo-VWD, is a rare inherited platelet disorder characterized by an increased affinity of the platelet membrane glycoprotein Ibalpha receptor for normal von Willebrand factor leading to characteristic platelet hyperaggregability. As PT-VWD shares most of the clinical and laboratory features of subtype 2B VWD, the differential diagnosis between these two inherited bleeding disorders requires either platelet-mixing or molecular genetic studies. In this review, the main clinical, laboratory and therapeutic characteristics of PT-VWD are concisely reported.

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Review Platelet-type von Willebrand disease and type 2B von Willebrand disease: a story of nonidentical twins when two different genetic abnormalities evolve into similar phenotypes. [Semin Thromb Hemost. 2007]
Review Platelet-type von Willebrand disease and type 2B von Willebrand disease: a story of nonidentical twins when two different genetic abnormalities evolve into similar phenotypes.
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Cited by 1 PubMed Central article

Diagnosis of platelet-type von Willebrand disease by flow cytometry. [Haematologica. 2010]
Diagnosis of platelet-type von Willebrand disease by flow cytometry.
Giannini S, Cecchetti L, Mezzasoma AM, Gresele P. Haematologica. 2010 Jun; 95(6):1021-4. Epub 2009 Nov 30.

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