Von Willebrand disease

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Última revisão das evidências: 19 Apr 2026

Última atualização do tópico: 11 Dec 2025

Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Type 1 VWD is more common and less severe than type 2 and 3 VWD.

VWD usually presents with mucocutaneous bleeding or bleeding after trauma or surgery. Heavy menstrual bleeding and postpartum hemorrhage are common in women with VWD. Joint bleeding is rare and usually only occurs in patients with severe disease (e.g., type 3 VWD).

Bleeding assessment tools (BATs) can help identify patients with VWD and guide referral for diagnostic testing.

Diagnosis requires blood tests to assess von Willebrand factor (VWF) antigen (VWF:Ag), platelet-dependent VWF activity (e.g., using VWF:GPIbM assay), and factor VIII coagulant activity (FVIII:C).

Management involves preventing and treating bleeding symptoms based on the specific type of VWD. Treatment options include desmopressin, VWF-containing concentrate, and antifibrinolytic therapy.

Definição

VWD is a bleeding disorder characterized by quantitative deficiency or qualitative defects of the VWF protein due to inherited mutations in the VWF gene.[1] VWF is critical for blood clotting as it facilitates platelet adhesion to exposed vascular subendothelium, and also binds and stabilizes coagulation factor VIII (FVIII).[1]

VWD is categorized into three main types (1, 2, and 3) based on whether the cause is a deficiency or defect of VWF.

Rarely, patients may develop acquired von Willebrand syndrome (AVWS) secondary to a lymphoproliferative, myeloproliferative, autoimmune, cardiovascular, or other disorder.[2][3][4] Details on the diagnosis and management of AVWS are beyond the scope of this topic.

História e exame físico

Principais fatores diagnósticos

family history of VWD and bleeding
easy and excessive bruising
bleeding from trauma or minor wounds
mucosal bleeding (e.g., epistaxis, gum bleeding)
heavy menstrual bleeding (HMB)
postpartum hemorrhage
postoperative bleeding

Detalhes completos

Outros fatores diagnósticos

gastrointestinal bleeding
history of repeat blood transfusions
joint bleeding (hemarthrosis)
hematuria
central nervous system bleeding

Detalhes completos

Fatores de risco

positive family history
consanguineous relationships

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

CBC
prothrombin time (PT)
activated partial thromboplastin time (aPTT)
von Willebrand factor (VWF) antigen (VWF:Ag)
von Willebrand factor (VWF) platelet-binding activity assay (VWF:GPIbM, VWF:GPIbR, or VWF:RCo)
factor VIII (FVIII) coagulant activity assay (FVIII:C)

Detalhes completos

Tests to avoid

bleeding time test

Detalhes completos

Investigações a serem consideradas

fibrinogen
thrombin time
von Willebrand factor (VWF) multimer analysis
von Willebrand factor (VWF) collagen binding assay (VWF:CB)
genetic testing (for type 2B or 2N VWD variants)
low-dose ristocetin-induced platelet agglutination (RIPA) mixing studies
von Willebrand factor (VWF) FVIII binding assay (VWF:FVIIIB)
VWF propeptide assay (VWFpp/VWF:Ag)
platelet function analyzer (e.g., PFA-100)

Detalhes completos

Algoritmo de tratamento

Inicial

VWD type unknown with bleeding

all types VWD with severe bleeding uncontrolled by other VWD-specific therapies

AGUDA

all types VWD with severe bleeding or before invasive procedures where sustained elevation of VWF levels is necessary: nonpregnant

minor bleeding or minor invasive procedures involving mucous membranes: nonpregnant

minor bleeding or minor invasive procedures not involving mucous membranes: nonpregnant

all types VWD: pregnant

CONTÍNUA

all types VWD with heavy menstrual bleeding

all types VWD with significant chronic or recurrent bleeding

Colaboradores

Autores

Craig Seaman, MD, MS

Assistant Professor of Medicine

Division of Hematology/Oncology

Associate Director

Hemophilia Center of Western Pennsylvania

Pittsburgh

Declarações

CS is a consultant for Takeda Pharmaceuticals.

Agradecimentos

Dr. Craig Seaman would like to gratefully acknowledge Professor Mike Laffan and Dr Barbara A. Konkle, previous contributors to this topic.

Declarações

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.

Revisores

Margaret Ragni, MD

Director

Hemophilia Center of Western Pennsylvania

Pittsburgh

Declarações

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

Declarações

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.

Créditos aos pareceristas

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As afiliações e declarações dos pareceristas referem--se ao momento da revisão.

Referências

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Principais artigos

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.Texto completo Resumo

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.Texto completo Resumo

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.Texto completo Resumo

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.Texto completo Resumo

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.Texto completo Resumo

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.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Mild hemophilia A
- Inherited platelet function disorder
- Acquired von Willebrand syndrome
Mais Diagnósticos diferenciais
Diretrizes
- 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
- MASAC recommendations on administration of vaccines to individuals with bleeding disorders
Mais Diretrizes
Folhetos informativos para os pacientes
Heavy periods
Mais Folhetos informativos para os pacientes
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Von Willebrand disease

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Tests to avoid

Investigações a serem consideradas

Algoritmo de tratamento

VWD type unknown with bleeding

all types VWD with severe bleeding uncontrolled by other VWD-specific therapies

all types VWD with severe bleeding or before invasive procedures where sustained elevation of VWF levels is necessary: nonpregnant

minor bleeding or minor invasive procedures involving mucous membranes: nonpregnant

minor bleeding or minor invasive procedures not involving mucous membranes: nonpregnant

all types VWD: pregnant

all types VWD with heavy menstrual bleeding

all types VWD with significant chronic or recurrent bleeding

Colaboradores

Autores

Craig Seaman, MD, MS

Declarações

Agradecimentos

Declarações

Revisores

Margaret Ragni, MD

Declarações

David Keeling, BSc, MD, FRCP, FRCPath

Declarações

Créditos aos pareceristas

Declarações

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Folhetos informativos para os pacientes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice