von Willebrand disease: laboratory aspects of diagnosis and treatment

被引:25
作者
Favaloro, EJ
Lillicrap, D
Lazzari, MA
Cattaneo, M
Mazurier, C
Woods, A
Meschengieser, S
Blanco, A
Kempfer, AC
Hubbard, A
Chang, A
机构
[1] Queens Univ, Richardson Lab, Dept Pathol & Lab Med, Kingston, ON, Canada
[2] Univ Milan, Osped San Paolo, Hematol Unit, Milan, Italy
[3] US FDA, Ctr Biol Evaluat & Res, Rockville, MD USA
关键词
factor concentrate; diagnosis; treatment; von Willebrand disease; von Willebrand factor;
D O I
10.1111/j.1365-2516.2004.00979.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
von Willebrand disease is the most common inherited bleeding disorder in humans. VWD can be classified into three major types, designated Types 1, 2 and 3; Type 2 can be further separated into subtypes 2A, 2B, 2M and 2N. The diagnosis of VWD requires a personal and family history of bleeding and confirmation by laboratory analysis. Although Types 2 and 3 are relatively straightforward to diagnose, there may be a risk of over-diagnosis of Type 1 because of an overlap within the normal range. We also report on the clinical profile and diagnosis of VWD in a South American cohort of patients and on the in vitro characteristics of some factor concentrates available for treatment of VWD.
引用
收藏
页码:164 / 168
页数:5
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