Last reviewed: 6 Apr 2021
Last updated: 29 Nov 2018



History and exam

Key diagnostic factors

  • bleeding from minor wounds
  • postoperative bleeding
  • family history of bleeding
  • easy and excessive bruising
  • menorrhagia

Other diagnostic factors

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

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)
  • CBC
  • 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 analyzers

Treatment algorithm


Professor of Haemostasis and Thrombosis

Imperial College Academic Health Sciences Centre

Hammersmith Hospital




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.


BAK declares that she has no competing interests.

Peer reviewersVIEW ALL


Hemophilia Center of Western Pennsylvania




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

Consultant Haematologist and Director

Oxford Haemophilia & Thrombosis Centre

Churchill Hospital




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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