作者: M. FRANCHINI , M. MONTAGNANA , G. LIPPI
DOI: 10.1111/J.1751-553X.2007.00978.X
关键词:
摘要: Platelet-type von Willebrand disease (PT-VWD), or pseudo-VWD, is a rare inherited platelet disorder characterized by an increased affinity of the membrane glycoprotein Ibalpha receptor for normal factor leading to characteristic hyperaggregability. As PT-VWD shares most clinical and laboratory features subtype 2B VWD, differential diagnosis between these two bleeding disorders requires either platelet-mixing molecular genetic studies. In this review, main clinical, therapeutic characteristics are concisely reported.