Nordic Haemophilia Council's Practical Guidelines on Diagnosis and Management of von Willebrand Disease

被引:23
作者
Lassila, Riitta [1 ]
Holme, Pal Andre [2 ]
Landorph, Andrea [3 ]
Petrini, Pia [4 ]
Onundarson, Pall T. [5 ,6 ]
Hillarp, Andreas [7 ,8 ]
机构
[1] Helsinki Univ Cent Hosp, Unit Coagulat Disorders, Dept Hematol & Clin Chem Lab Serv, Helsinki, Finland
[2] Oslo Univ Hosp, Dept Hematol, Oslo, Norway
[3] Rigshosp, Thrombosis & Hemostasis Unit, DK-2100 Copenhagen, Denmark
[4] Karolinska Univ Hosp, Astrid Lindgren Childrens Hosp, Coagulat Unit Children, Stockholm, Sweden
[5] Dept Lab Hematol & Coagulat Disorders Landspitali, Reykjavik, Iceland
[6] Univ Iceland, Sch Med, Reykjavik, Iceland
[7] Univ Malmo, Malmo Ctr Thrombosis & Haemostasis, Malmo, Sweden
[8] Malmo Univ Hosp, Reg Labs Reg Scania, Malmo, Sweden
关键词
von Willebrand disease; Nordic Haemophilia Council; Guidelines; COLLAGEN-BINDING ASSAY; BLEEDING SYMPTOMS; CLINICAL MANIFESTATIONS; MOLECULAR-BIOLOGY; TYPE-1; VWD; DESMOPRESSIN; PATHOPHYSIOLOGY; IDENTIFICATION; MULTICENTER;
D O I
10.1055/s-0031-1281034
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
von Willebrand disease (VWD) is the most common inherited bleeding disorder characterized by spontaneous or tissue injury related, mostly mucocutaneous, bleeding events. VWD affects both males and females and is caused by quantitative or qualitative deficiency of von Willebrand factor. The diagnostic procedure is complicated because VWD is highly heterogeneous, and differential diagnosis from platelet disorders may be challenging. Moreover, these defects may even coexist, impacting the bleeding phenotype. Mild and moderate VWD can be difficult to distinguish from the normal population, and VWD subtyping may also be problematic. This article summarizes the guidelines of the Nordic Haemophilia Council (NHC), which are intended to serve as a practical tool and provide the standards for diagnosing and treating VWD patients. The complete Nordic Guidelines on VWD are available at the NHC Web site (http://nordhemophilia.org).
引用
收藏
页码:495 / 502
页数:8
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