The Unrecognized von Willebrand Disease: A Frequent Cause of Bleeding Complications

被引：0
|
作者：
Schneppenheim, Reinhard [1 ]
Budde, Ulrich [1 ]
机构：
[1] Med Ctr Hamburg Eppendorf, Dept Pediat Hematol & Oncol, Hamburg, Germany
来源：
PROGRESS AND CHALLENGES IN TRANSFUSION MEDICINE, HEMOSTASIS AND HEMOTHERAPY: STATE OF THE ART 2008 | 2008年
关键词：
von Willebrand disease; von Willebrand factor; Platelets; Collagen; ADAMTS13;
D O I：
10.1159/000177250
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
Quantitative and qualitative defects of von Willebrand factor (VWF) cause von Willebrand disease (VWD), the most common inborn bleeding disorder being inherited in a mainly autosomal dominant but also recessive manner. According to its modular structure characterized by distinct functional and structural domains, VWF defects correlate with considerable heterogeneity of clinical symptoms, biochemical parameters and of the underlying molecular mechanisms. This renders the diagnostic approach demanding and is also the reason for under- or misdiagnosis of VWD. (C) 2008 S. Karger GmbH, Freiburg i.Br.
引用
收藏
页码：288 / 295
页数：8
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