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Clinical, laboratory and therapeutic aspects of platelet‐type von Willebrand disease
Author(s) -
FRANCHINI M.,
MONTAGNANA M.,
LIPPI G.
Publication year - 2008
Publication title -
international journal of laboratory hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.705
H-Index - 55
eISSN - 1751-553X
pISSN - 1751-5521
DOI - 10.1111/j.1751-553x.2007.00978.x
Subject(s) - von willebrand disease , platelet , platelet disorder , von willebrand factor , platelet membrane glycoprotein , medicine , immunology
Summary 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 Ibα 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.