Last reviewed: 20 Sep 2021
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

Other diagnostic factors

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

Risk factors

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

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

Investigations to consider

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

Emerging tests

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

Treatment algorithm

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.

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