Journal
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
Volume 30, Issue 2, Pages 91-94Publisher
WILEY
DOI: 10.1111/j.1751-553X.2007.00978.x
Keywords
platelet-type VWD; pseudo VWD; bleeding; therapy
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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 Ib alpha 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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