Doença de von Willebrand
Summary
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- história familiar de DVW e sangramento
- hematomas excessivos ou facilidade de adquiri-los
- sangramento devido a trauma e pequenas feridas
- sangramento da mucosa (por exemplo, epistaxe, sangramento gengival)
- sangramento menstrual intenso (SMI)
- hemorragia pós-parto
- sangramento pós-operatório
Other diagnostic factors
- hemorragia digestiva
- história de transfusões de sangue repetidas
- sangramento articular (hemartrose)
- hematúria
- sangramento no sistema nervoso central
Risk factors
- história familiar positiva
- relacionamentos consanguíneos
Diagnostic tests
1st tests to order
- Hemograma completo
- tempo de protrombina (TP)
- tempo de tromboplastina parcial ativada (TTPa)
- antígeno do fator de von Willebrand (FvW) (FvW:Ag)
- ensaio de atividade de ligação plaquetária do fator de von Willebrand (FvW:GPIbM, FvW:GPIbR ou FvW:RCo)
- ensaio de atividade coagulante do fator VIII (FVIII) (FVIII:C)
Tests to consider
- fibrinogênio
- tempo de trombina
- análise do multímero do fator de von Willebrand (FvW)
- ensaio de ligação do fator de von Willebrand (FvW) ao colágeno (FvW:CB)
- teste genético (para variantes 2B ou 2N da DVW)
- estudos mistos de aglutinação plaquetária induzida por ristocetina (RIPA) em baixa dose
- ensaio de ligação do fator de von Willebrand (FvW) do FVIII (FvW:FVIIIB)
- Ensaio de pró-peptídeo do FvW (FvWpp/FvW:Ag)
- analisador de função plaquetária (por exemplo, PFA-100)
Treatment algorithm
DVW de tipo desconhecido com sangramento
todos os tipos de DVW com sangramento grave não controlado por outras terapias específicas para a DVW
todos os tipos de DVW com sangramento grave ou antes de procedimentos invasivos onde a elevação sustentada dos níveis de FvW é necessária: não gestantes
sangramento mínimo ou pequenos procedimentos invasivos envolvendo membranas mucosas: não gestante
sangramento mínimo ou pequenos procedimentos invasivos não envolvendo membranas mucosas: não gestante
DVW de todos os tipos: gestante
todos os tipos de DVW com sangramento menstrual intenso
todos os tipos de DVW com sangramento crônico ou recorrente significativo
Contributors
Authors
Craig Seaman, MD, MS
Assistant Professor of Medicine
Division of Hematology/Oncology
Associate Director
Hemophilia Center of Western Pennsylvania
Pittsburgh
PA
Disclosures
CS is a consultant for Takeda Pharmaceuticals.
Acknowledgements
Dr. Craig Seaman would like to gratefully acknowledge Professor Mike Laffan and Dr Barbara A. Konkle, previous contributors to this topic.
Disclosures
ML declares he has received consultancy fees from Pfizer, Takeda, Sobi, CSL Behring, AstraZeneca, and Roche; and speaker fees from: Pfizer, Takeda, CSL Behring, Sobi, AstraZeneca, Leo Pharma, and Bayer; and travel support from Bayer and Shire. ML is an author of several references cited in this topic. 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.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor. J Thromb Haemost. 2006 Oct;4(10):2103-14.Full text Abstract
Pavord S, Rayment R, Madan B, et al; on behalf of the Royal College of Obstetricians and Gynaecologists. Management of inherited bleeding disorders in pregnancy. Green-top guideline No 71 (joint with UKHCDO). BJOG. 2017 Jul;124(8):e193–263.Full text Abstract
James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):280-300.Full text Abstract
Laffan MA, Lester W, O'Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014 Nov;167(4):453-65.Full text Abstract
Platton S, Baker P, Bowyer A, et al. Guideline for laboratory diagnosis and monitoring of von Willebrand disease: a joint guideline from the United Kingdom Haemophilia Centre Doctors' Organisation and the British Society for Haematology. Br J Haematol. 2024 May;204(5):1714-31.Full text Abstract
Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):301-25.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Hemofilia A leve
- Distúrbio de função plaquetária hereditário
- Síndrome de von Willebrand adquirida
More DifferentialsGuidelines
- Guideline for laboratory diagnosis and monitoring of von Willebrand disease: A joint guideline from the United Kingdom Haemophilia Centre Doctors' Organisation and the British Society for Haematology
- Recommendations for management of type 3 von Willebrand disease in Australia
More GuidelinesPatient information
Menstruação intensa
More Patient information
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