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

被引：17
|
作者：
Franchini, M. [1 ]
Montagnana, M. [2 ]
Lippi, G. [2 ]
机构：
[1] Azienda Osped Verona, Serv Immunoematol & Trasfus, Ctr Emofilia, Verona, Italy
[2] Univ Verona, Ist Chim & Microscopia Clin, Dipartimento Sci Biomed & Morfol, I-37100 Verona, Italy
来源：
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY | 2008年 / 30卷 / 02期
关键词：
platelet-type VWD; pseudo VWD; bleeding; therapy;
D O I：
10.1111/j.1751-553X.2007.00978.x
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
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 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.
引用
收藏
页码：91 / 94
页数：4
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