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Von Willebrand disease

  • 概述
  • 理论
  • 诊断
  • 治疗
  • 随访
  • 资源
最后审阅： 29 May 2026
最后更新： 11 Dec 2025

小结

定义

病史和体格检查

关键诊断因素

  • family history of VWD and bleeding
  • easy and excessive bruising
  • bleeding from trauma or minor wounds
  • mucosal bleeding (e.g., epistaxis, gum bleeding)
  • heavy menstrual bleeding (HMB)
  • postpartum hemorrhage
  • postoperative bleeding
完整详情

其他诊断因素

  • gastrointestinal bleeding
  • history of repeat blood transfusions
  • joint bleeding (hemarthrosis)
  • hematuria
  • central nervous system bleeding
完整详情

危险因素

  • positive family history
  • consanguineous relationships
完整详情

诊断性检查

首要检查

  • CBC
  • prothrombin time (PT)
  • activated partial thromboplastin time (aPTT)
  • von Willebrand factor (VWF) antigen (VWF:Ag)
  • von Willebrand factor (VWF) platelet-binding activity assay (VWF:GPIbM, VWF:GPIbR, or VWF:RCo)
  • factor VIII (FVIII) coagulant activity assay (FVIII:C)
完整详情

Tests to avoid

  • bleeding time test
完整详情

需考虑的检查

  • fibrinogen
  • thrombin time
  • von Willebrand factor (VWF) multimer analysis
  • von Willebrand factor (VWF) collagen binding assay (VWF:CB)
  • genetic testing (for type 2B or 2N VWD variants)
  • low-dose ristocetin-induced platelet agglutination (RIPA) mixing studies
  • von Willebrand factor (VWF) FVIII binding assay (VWF:FVIIIB)
  • VWF propeptide assay (VWFpp/VWF:Ag)
  • platelet function analyzer (e.g., PFA-100)
完整详情

治疗流程

初步治疗

VWD type unknown with bleeding

all types VWD with severe bleeding uncontrolled by other VWD-specific therapies

急症处理

all types VWD with severe bleeding or before invasive procedures where sustained elevation of VWF levels is necessary: nonpregnant

minor bleeding or minor invasive procedures involving mucous membranes: nonpregnant

minor bleeding or minor invasive procedures not involving mucous membranes: nonpregnant

all types VWD: pregnant

持续性治疗

all types VWD with heavy menstrual bleeding

all types VWD with significant chronic or recurrent bleeding

撰稿人

作者

Craig Seaman, MD, MS

Assistant Professor of Medicine

Division of Hematology/Oncology

Associate Director

Hemophilia Center of Western Pennsylvania

Pittsburgh

PA

利益声明

CS is a consultant for Takeda Pharmaceuticals.

鸣谢

Dr. Craig Seaman would like to gratefully acknowledge Professor Mike Laffan and Dr Barbara A. Konkle, previous contributors to this topic.

利益声明

ML declares he has received consultancy fees from Pfizer, Takeda, Sobi, CSL Behring, AstraZeneca, and Roche; and speaker fees from: Pfizer, Takeda, CSL Behring, Sobi, AstraZeneca, Leo Pharma, and Bayer; and travel support from Bayer and Shire. ML is an author of several references cited in this topic. BAK declares that she has no competing interests.

同行评议者

Margaret Ragni, MD

Director

Hemophilia Center of Western Pennsylvania

Pittsburgh

PA

利益声明

MR is an author of a reference cited in this topic.

David Keeling, BSc, MD, FRCP, FRCPath

Consultant Haematologist and Director

Oxford Haemophilia & Thrombosis Centre

Churchill Hospital

Oxford

UK

利益声明

DK declares that he has received payments from CSL Behring for giving a lecture and attending an advisory board. He is an author of a number of references cited in this topic.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

参考文献

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

关键文献

Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor. J Thromb Haemost. 2006 Oct;4(10):2103-14.全文  摘要

Pavord S, Rayment R, Madan B, et al; on behalf of the Royal College of Obstetricians and Gynaecologists. Management of inherited bleeding disorders in pregnancy. Green-top guideline No 71 (joint with UKHCDO). BJOG. 2017 Jul;124(8):e193–263.全文  摘要

James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):280-300.全文  摘要

Laffan MA, Lester W, O'Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014 Nov;167(4):453-65.全文  摘要

Platton S, Baker P, Bowyer A, et al. Guideline for laboratory diagnosis and monitoring of von Willebrand disease: a joint guideline from the United Kingdom Haemophilia Centre Doctors' Organisation and the British Society for Haematology. Br J Haematol. 2024 May;204(5):1714-31.全文  摘要

Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):301-25.全文  摘要

参考文献

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

  • 鉴别诊断

    • Mild hemophilia A
    • Inherited platelet function disorder
    • Acquired von Willebrand syndrome
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  • 指南

    • Guideline for laboratory diagnosis and monitoring of von Willebrand disease: a joint guideline from the United Kingdom Haemophilia Centre Doctors' Organisation and the British Society for Haematology
    • MASAC recommendations on administration of vaccines to individuals with bleeding disorders
    更多 指南
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