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Last reviewed: 21 Apr 2025
Last updated: 29 Nov 2018

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • bleeding from minor wounds
  • postoperative bleeding
  • family history of bleeding
  • easy and excessive bruising
  • menorrhagia
Full details

Other diagnostic factors

  • gastrointestinal bleeding
  • epistaxis
  • blood transfusions
  • haemarthrosis
  • central nervous system bleeding
  • haematuria
Full details

Risk factors

  • positive family history
  • consanguineous relationships
  • lymphoproliferative disorders
  • aortic stenosis
  • myeloproliferative disorders
  • hypothyroidism
Full details

Diagnostic investigations

1st investigations to order

  • prothrombin time (PT)
  • activated partial thromboplastin time (APTT)
  • FBC
  • von Willebrand factor antigen
  • von Willebrand factor function assay (ristocetin cofactor and collagen binding assays)
  • factor VIII activity
Full details

Investigations to consider

  • von Willebrand factor multimer analysis
  • platelet aggregometry
  • factor VIII - von Willebrand factor binding assay
  • TFTs
  • serum protein electrophoresis
Full details

Emerging tests

  • mutation analysis
  • PFA-100 and other platelet function analysers
Full details

Treatment algorithm

INITIAL

VWD type unknown with active severe haemorrhage

all types VWD with severe bleeding uncontrolled by desmopressin, antifibrinolytics, and von Willebrand factor-containing concentrates

ACUTE

all types VWD with severe bleeding or before high-risk bleeding procedures (including where sustained high levels of VWF required for several days): with or without mucosal involvement

all types VWD with minor bleeding or before procedures: involving mucous membranes

type 1, 2A, or 2M VWD: desmopressin responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant

type 1, 2A, or 2M VWD: desmopressin non-responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant

type 3, 2B, or 2N VWD: all bleeding or procedures except minor mucosal bleeding or before minor procedures involving mucosal surfaces: non-pregnant

all types VWD: pregnant

ONGOING

all types VWD with chronic or recurrent menorrhagia

all types VWD with significant chronic or recurrent bleeding or with treatment-refractory menorrhagia

Contributors

Authors

Mike Laffan, DM, FRCP, FRCPath

Professor of Haemostasis and Thrombosis

Imperial College Academic Health Sciences Centre

Hammersmith Hospital

London

UK

Disclosures

ML has received consultancy fees from Pfizer, Shire, Portola and Roche, speaker fees from Pfizer and Bayer, and travel support from Bayer and Shire. ML is an author of a number of references cited in this topic.

Acknowledgements

Prof Mike Laffan would like to gratefully acknowledge Dr Barbara A. Konkle, a previous contributor to this topic.

Disclosures

BAK declares that she has no competing interests.

Peer reviewers

Margaret Ragni, MD

Director

Hemophilia Center of Western Pennsylvania

Pittsburgh

PA

Disclosures

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

Disclosures

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.

References

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.

Key articles

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.Full text  Abstract

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.Full text  Abstract

Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 vWD). J Thromb Haemost. 2006 Apr;4(4):766-73.Full text  Abstract

Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders: a United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline approved by the British Committee for Standards in Haematology. Haemophilia. 2008 Jul;14(4):671-84.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available here.

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