Von Willebrand disease

Last reviewed: 27 Apr 2022
Last updated: 29 Nov 2018

Summary

Definition

History and exam

Key diagnostic factors

  • bleeding from minor wounds
  • postoperative bleeding
  • family history of bleeding
  • easy and excessive bruising
  • menorrhagia
More key diagnostic factors

Other diagnostic factors

  • gastrointestinal¬†bleeding
  • epistaxis
  • blood transfusions
  • hemarthrosis
  • central¬†nervous system bleeding
  • hematuria
Other diagnostic factors

Risk factors

  • positive family history
  • consanguineous relationships
  • lymphoproliferative disorders
  • aortic stenosis
  • myeloproliferative disorders
  • hypothyroidism
More risk factors

Diagnostic investigations

1st investigations to order

  • prothrombin time (PT)
  • activated partial thromboplastin time (APTT)
  • CBC
  • von Willebrand factor antigen
  • von Willebrand factor function assay (ristocetin cofactor and collagen binding assays)
  • factor VIII activity
More 1st investigations to order

Investigations to consider

  • von Willebrand factor multimer analysis
  • platelet aggregometry
  • factor VIII - von Willebrand factor binding assay
  • TFTs
  • serum protein electrophoresis
More investigations to consider

Emerging tests

  • mutation analysis
  • PFA-100 and other platelet function analyzers

Treatment algorithm

INITIAL

VWD type unknown with active severe hemorrhage

all types VWD with severe bleeding uncontrolled by desmopressin, antifibrinolytics, and VWF-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 nonmucosal bleeding or before procedures not involving mucous membranes: nonpregnant

type 1, 2A, or 2M VWD: desmopressin nonresponder with moderate mucosal or minor/moderate nonmucosal bleeding or before procedures not involving mucous membranes: nonpregnant

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

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.

  • Differentials

    • Mild hemophilia A
    • Inherited platelet function disorder
    More Differentials
  • Guidelines

    • Management of inherited bleeding disorders in pregnancy
    • The diagnosis and management of von Willebrand disease
    More Guidelines
  • Patient leaflets

    Heavy periods

    More Patient leaflets
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