Last reviewed: September 2018
Last updated: February  2017



History and exam

Key diagnostic factors

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

Other diagnostic factors

  • GI bleeding
  • epistaxis
  • blood transfusions
  • hemarthrosis
  • CNS 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
  • VWF antigen
  • VWF function assay (ristocetin cofactor and collagen binding assays)
  • factor VIII activity
Full details

Investigations to consider

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

Emerging tests

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

Treatment algorithm


Authors VIEW ALL

Professor of Haemostasis and Thrombosis

Imperial College Academic Health Sciences Centre

Hammersmith Hospital




ML has received consultancy and lecture fees from Pfizer, CSL-Behring, and LFB; research support from Bayer; and travel support from LFB and Pfizer. ML is an author of a number of references cited in this monograph.

Prof Mike Laffan would like to gratefully acknowledge Dr Barbara A. Konkle, a previous contributor to this monograph. BAK declares that she has no competing interests.

Peer reviewers VIEW ALL


Hemophilia Center of Western Pennsylvania




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

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 monograph.

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