How I manage severe von Willebrand disease

被引：29

作者：

Leebeek, Frank W. G. ^{[1
]}

Atiq, Ferdows ^{[1
]}

机构：

[1] Erasmus Univ, Med Ctr, Dept Haematol, POB 2040, NL-3000 CA Rotterdam, Netherlands

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2019年 / 187卷 / 04期

关键词：

von Willebrand disease; treatment; severe VWD; QUALITY-OF-LIFE; INHERITED BLEEDING DISORDERS; NORMAL PLATELET TRANSFUSION; GENE-THERAPY; REPLACEMENT THERAPY; THROMBUS FORMATION; ADULT PATIENTS; JOINT BLEEDS; DIAGNOSIS; ALLOANTIBODIES;

D O I：

10.1111/bjh.16186

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. Most patients with mild and moderate VWD can be treated effectively with desmopressin. The management of severe VWD patients, mostly affected by type 2 and type 3 disease, can be challenging. In this article we review the current diagnosis and treatment of severe VWD patients. We will also discuss the management of severe VWD patients in specific situations, such as pregnancy, delivery, patients developing alloantibodies against von Willebrand factor and VWD patients with recurrent gastrointestinal bleeding. Moreover, we review emerging treatments that may be applied in future management of patients with severe VWD.

引用

页码：418 / 430

页数：13

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