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
VWD type unknown with active severe haemorrhage
all types VWD with severe bleeding uncontrolled by desmopressin, antifibrinolytics, and von Willebrand factor-containing concentrates
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 non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant
type 1, 2A, or 2M VWD: desmopressin non-responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant
type 3, 2B, or 2N VWD: all bleeding or procedures except minor mucosal bleeding or before minor procedures involving mucosal surfaces: non-pregnant
all types VWD: pregnant
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 haemophilia A
- Inherited platelet function disorder
More DifferentialsGuidelines
- Management of inherited bleeding disorders in pregnancy
- The diagnosis and management of von Willebrand disease
More GuidelinesPatient information
Heavy periods
More Patient information
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