Last reviewed: June 2019
Last updated: November  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
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

Contributors

Authors VIEW ALL

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.

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

Peer reviewers VIEW ALL

Director

Hemophilia Center of Western Pennsylvania

Pittsburgh

PA

Disclosures

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

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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