Raras
Buscar doenças, sintomas, genes...
Doença de von Willebrand adquirida
ORPHA:99147CID-10 · D68.4CID-11 · 3B2YDOENÇA RARA

A síndrome de von Willebrand adquirida (SAVA) é um distúrbio hemorrágico marcado pelas mesmas anomalias biológicas observadas na doença hereditária de von Willebrand (DVW), mas que ocorre em associação com outra patologia subjacente, geralmente em pacientes idosos sem qualquer história pessoal ou familiar de anomalias hemorrágicas.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A síndrome de von Willebrand adquirida (SAVA) é um distúrbio hemorrágico marcado pelas mesmas anomalias biológicas observadas na doença hereditária de von Willebrand (DVW), mas que ocorre em associação com outra patologia subjacente, geralmente em pacientes idosos sem qualquer história pessoal ou familiar de anomalias hemorrágicas.

Pesquisas ativas
2 ensaios
7 total registrados no ClinicalTrials.gov
Publicações científicas
599 artigos
Último publicado: 2026
Medicamentos
1 registrados
VON WILLEBRAND FACTOR HUMAN

Tem tratamento?

1 medicamento registrado
Ver detalhes, fases e interações →
VON WILLEBRAND FACTOR HUMAN

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
300
pacientes catalogados
Início
Adult
🏥
SUS: Cobertura mínimaScore: 20%
1 medicamentos CEAFCID-10: D68.4
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
6 sintomas
❤️
Coração
4 sintomas
🦴
Ossos e articulações
2 sintomas
🧬
Pele e cabelo
1 sintomas
🫘
Rins
1 sintomas
🫃
Digestivo
1 sintomas

+ 12 sintomas em outras categorias

Características mais comuns

55%prev.
Neoplasia hematológica
Frequente (79-30%)
55%prev.
Morfologia anormal do sistema cardiovascular
Frequente (79-30%)
55%prev.
Atividade reduzida do fator VIII
Frequente (79-30%)
55%prev.
Atividade prejudicada do ensaio do cofator de ristocetina
Frequente (79-30%)
55%prev.
Tempo de protrombina prolongado
Frequente (79-30%)
55%prev.
Atividade reduzida do fator de von Willebrand
Frequente (79-30%)
27sintomas
Frequente (6)
Ocasional (20)
Muito raro (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.

Neoplasia hematológicaHematological neoplasm
Frequente (79-30%)55%
Morfologia anormal do sistema cardiovascularAbnormal cardiovascular system morphology
Frequente (79-30%)55%
Atividade reduzida do fator VIIIReduced factor VIII activity
Frequente (79-30%)55%
Atividade prejudicada do ensaio do cofator de ristocetinaImpaired ristocetin cofactor assay activity
Frequente (79-30%)55%
Tempo de protrombina prolongadoProlonged prothrombin time
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico599PubMed
Últimos 10 anos200publicações
Pico202251 papers
Linha do tempo
2026Hoje · 2026🧪 2008Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
2Fase 22
·Pré-clínico3
Medicamentos catalogadosEnsaios clínicos· 1 medicamento · 5 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Doença de von Willebrand adquirida

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

7 ensaios clínicos encontrados, 2 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Divulgue para pacientes e familiares que acompanham esta doença.
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Publicações mais relevantes

Timeline de publicações
385 papers (10 anos)
#1

Heyde Syndrome: A Literature Review.

Health science reports2026 Feb

Heyde syndrome, a triad of anemia from gastrointestinal (GI) bleeding, aortic valve stenosis, and acquired von Willebrand syndrome, primarily affects individuals over 65. Management requires a multidisciplinary approach, including medical therapy, endoscopic intervention, and valve replacement. Despite advances, many aspects remain unclear. This review explores the epidemiology, pathophysiology, and management of Heyde syndrome while highlighting areas for future research. A comprehensive search of PubMed and Google Scholar focused on English-language human studies, including case reports, clinical trials, reviews, and expert guidelines. Additional literature on Von Willebrand syndrome and GI angiodysplasia was reviewed. The final search was completed on April 5, 2025. Aortic valve replacement improves outcomes and hematologic abnormalities. Transcatheter aortic valve replacement (TAVR) showed lower rates of life-threatening bleeding, fewer perioperative complications, and reduced transfusions compared to surgical replacement. Guidelines recommend dual antiplatelet therapy (DAPT) for 3-6 months post-TAVR, with 79%-86% of patients experiencing no recurrence of GI bleeds. Management of intestinal angiodysplasia often shows high recurrence rates due to persistent lesions and vWS-2A. Surgical valve replacement also improves bleeding control, even with anticoagulation. Aortic valve replacement should be first-line therapy in severe aortic stenosis with chronic GI bleeding. Heyde syndrome remains a complex and often underrecognized condition in elderly populations. Continued research is critical to deepen understanding and improve patient outcomes, allowing for more personalized and effective care strategies.

#2

Thrombotic and Bleeding Complications in Myeloproliferative Neoplasms: An Integrated Clinical Perspective.

Seminars in thrombosis and hemostasis2026 Mar 19

Myeloproliferative neoplasms (MPNs) are clonal hematopoietic disorders characterized by elevated thrombotic and bleeding risk, and optimal risk stratification and management remain challenging. This review summarizes current evidence on the thrombotic complications in MPNs, including venous events (i.e. deep-vein thrombosis, pulmonary embolism and unusual site thrombosis), and arterial events (ischemic stroke, myocardial infarction and peripheral arterial thrombosis) and the increased bleeding risk in these diseases. Mechanistically, JAK2-driven clonal hematopoiesis, elevated hematocrit, leukocytosis, platelet activation, endothelial dysfunction and chronic inflammation interact to promote a pro-thrombotic state; conversely, extreme thrombocytosis, acquired von Willebrand syndrome (aVWS) and anticoagulant/antiplatelet therapy contribute to bleeding risk. Clinically, thrombosis may precede MPN diagnosis, especially in unusual sites, and treatment should balance the risk of recurrent thrombosis against the risk of hemorrhagic complications. Antithrombotic strategies include low-dose aspirin, vitamin K antagonists and direct oral anticoagulants, while cytoreductive therapy (hydroxyurea, anagrelide, interferon and JAK inhibitors) is central for disease control as well as vascular risk reduction. Despite therapy, recurrence of thrombotic events and major bleeding persists, highlighting the need for optimized risk models and alternative therapeutic targets. Future research may focus on integrating molecular biomarkers, inflammation metrics and vascular-specific endpoints to direct personalized preventive strategies.

#3

Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.

Journal of thrombosis and haemostasis : JTH2026 Mar 16

Monoclonal gammopathies of clinical significance represent a heterogeneous spectrum of non-malignant disorders that may involve multiple organ systems or remain organ-restricted. These conditions are driven by circulating monoclonal proteins (MIg) secreted by small, often indolent, B-cell or plasma cell clones. Accumulating evidence has demonstrated that MIg may possess both thrombogenic and hemorrhagic potential, mediated through complex and multifactorial interactions with coagulation pathways, platelets, and the vascular endothelium. In this review, we critically appraise the spectrum of hemostatic complications associated with monoclonal gammopathies, encompassing entities such as amyloidosis, cold agglutinin disease, M-protein-associated antiphospholipid antibody syndrome, anti-PF4 antibody-mediated disorders, and thrombotic microangiopathies, among others. Given their substantial clinical burden and frequent under recognition, we propose that these MIg-driven hemostatic disorders be classified separately as monoclonal gammopathies of thrombotic/hemorrhagic significance (MGTHS) encompassing both thrombotic and hemorrhagic subtypes. We additionally outline a proposed diagnostic approach emphasizing systematic evaluation for alternative etiologies, characterization of the monoclonal protein, and assessment for clinical, laboratory, and treatment-response features supporting pathogenic attribution. Establishing such a nosological framework highlights the clinical importance of these conditions and underscores the need for systematic recognition, diagnostic evaluation, and multidisciplinary management of these often overlooked but clinically consequential entities.

#4

Efficacy of venetoclax-obinutuzumab treatment in achieving remission of acquired von Willebrand syndrome in chronic lymphocytic leukemia.

Annals of hematology2026 Mar 14

Venetoclax-obinutuzumab therapy successfully induced remission of acquired von Willebrand syndrome (AVWS) in a 72-year-old patient with chronic lymphocytic leukemia (CLL). Initially diagnosed with CLL, the patient later experienced severe bleeding due to AVWS, as evidenced by reduced factor VIII (FVIII) and von Willebrand factor (VWF) levels. Despite initial treatment with VWF/FVIII concentrates and tranexamic acid, persistent bleeding prompted the initiation of venetoclax-obinutuzumab. This regimen resulted in normalization of coagulation parameters and undetectable minimal residual disease, indicating effective CLL control and subsequent AVWS remission. This case highlights the potential of targeted CLL therapies in the management of rare associated hematologic complications.

#5

Heyde syndrome secondary to hypertrophic obstructive cardiomyopathy: a case report.

The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology2026 Mar 09

Heyde syndrome is an uncommon clinical condition characterized by the triad of aortic stenosis, gastrointestinal angiodysplasia, and acquired von Willebrand syndrome. Its occurrence in the context of hypertrophic obstructive cardiomyopathy is rare. Common in individuals over 65, diagnosis is challenging due to the prevalence of these conditions. Hypertrophic obstructive cardiomyopathy increases shear stress, leading to von Willebrand Factor degradation and a higher risk of gastrointestinal bleeding. A 69-year-old male presented to the outpatient clinic with melena, abdominal cramping, and fatigue, along with a history of anemia requiring blood transfusions. Despite a negative upper gastrointestinal endoscopy, further tests revealed angiodysplasias with recent bleeding. He was also diagnosed with hypertrophic cardiomyopathy with left ventricular outflow tract obstruction. After being treated with octreotide and nadolol, his condition improved markedly, with hemoglobin levels rising and no further episodes of bleeding. Recognizing hypertrophic obstructive cardiomyopathy as a new variant of Heyde syndrome broadens our understanding of this complex disorder. It emphasizes the need to consider hypertrophic obstructive cardiomyopathy in patients with unexplained gastrointestinal bleeding and cardiac murmurs, prompting a more tailored approach to diagnosis and treatment.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC347 artigos no totalmostrando 198

2026

Thrombotic and Bleeding Complications in Myeloproliferative Neoplasms: An Integrated Clinical Perspective.

Seminars in thrombosis and hemostasis
2026

Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.

Journal of thrombosis and haemostasis : JTH
2026

Efficacy of venetoclax-obinutuzumab treatment in achieving remission of acquired von Willebrand syndrome in chronic lymphocytic leukemia.

Annals of hematology
2026

Heyde syndrome secondary to hypertrophic obstructive cardiomyopathy: a case report.

The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology
2026

von Willebrand factor in hemodialysis patients. Does acquired von Willebrand syndrome occur?

Thrombosis research
2026

von Willebrand factor dynamics in trauma managed with extracorporeal life support.

Journal of thrombosis and haemostasis : JTH
2026

Heyde Syndrome: A Literature Review.

Health science reports
2025

Budd-Chiari Syndrome Secondary to Essential Thrombocythaemia Complicated by Acquired Von Willebrand Disease and Mimicking Hepatic Malignancy: A Case Report.

Cureus
2025

Effect of Pulse Frequency on Shear-Induced Extension of Von Willebrand Factor in Continuous-Flow Ventricular Assist Device (CF-VAD).

ASAIO journal (American Society for Artificial Internal Organs : 1992)
2025

Acquired von Willebrand Syndrome: A Comprehensive Review and a Nordic Perspective.

Seminars in thrombosis and hemostasis
2025

[Diffuse large B-cell lymphoma with acquired von Willebrand syndrome: a case report and literature review].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2026

Anticoagulation and hemostasis in the extracorporeal membrane oxygenation patient.

Current opinion in anaesthesiology
2025

Heyde's Syndrome: Presentation and Successful Treatment With Transcatheter Aortic Valve Implantation (TAVI).

Cureus
2025

Heterogeneous Hemostatic Disorder Associated with IgA Monoclonal Gammopathy: A Case Series.

Endocrine, metabolic &amp; immune disorders drug targets
2025

Concomitant Acquired Hemophilia A and Acquired Von Willebrand Syndrome from Distinctive Autoantibodies: Case Report.

Hematology reports
2025

Acquired von Willebrand syndrome in children.

Research and practice in thrombosis and haemostasis
2025

Managing High Risk Pregnancy in Single Ventricle Physiology with Acquired von Willebrand Disease: A Case Report.

Reports (MDPI)
2025

Stroke in Mechanical Circulatory Supported Cardiogenic Shock.

Thrombosis and haemostasis
2025

Acquired von Willebrand syndrome in young children with congenital heart defects: focus on patent ductus arteriosus and ventricular septal defect.

Research and practice in thrombosis and haemostasis
2025

Successful Treatment of Acquired von Willebrand Syndrome in a Rare Case of Nodal Marginal Zone Lymphoma With Rituximab Therapy: A Case Report.

Cureus
2025

Complications at Diagnosis of Pediatric Chronic Myeloid Leukemia in Chronic Phase.

Pediatric blood &amp; cancer
2025

Thrombocytosis and bleeding in myeloproliferative neoplasms: exploring clinical diversity and risk of acquired von Willebrand syndrome-insights from a UK center.

Research and practice in thrombosis and haemostasis
2025

VON WILLEBRAND FACTOR IN ECMO: A DYNAMIC MODULATOR OF HEMORRHAGE AND THROMBOSIS.

Shock (Augusta, Ga.)
2025

Bleeding Complications in JAK2-Variant Essential Thrombocythemia: A Revisit in 2025.

EJHaem
2025

Acquired von Willebrand syndrome secondary to monoclonal gammopathy: a single-center case series.

Therapeutic advances in hematology
2025

Von Willebrand factor is a multifaceted player in hemostasis requiring a diverse array of analytical and diagnostic approaches.

Expert review of hematology
2025

Wilms Tumor and Paraneoplastic Syndromes: Unusual Presentation With Acquired von Willebrand Disease, Polycythemia and Hyperviscosity-A Comprehensive Case Report and Literature Reviews.

Journal of pediatric hematology/oncology
2025

Bleeding due to a Broken Heart Valve: Heyde's Syndrome Secondary to Severe Mitral Regurgitation.

Gastro hep advances
2025

Pre-procedural abnormal von Willebrand factor function predicts clinical outcomes after Transcatheter Aortic Valve Implantation: a prospective cohort study.

Frontiers in cardiovascular medicine
2025

Management of COVID-19 associated coagulopathy in critically ill patients and the risk of acquired von willebrand syndrome.

Scientific reports
2025

Perioperative management of transcatheter aortic valve implantation in acquired von Willebrand syndrome secondary to monoclonal gammopathy: a case report.

European heart journal. Case reports
2025

Heyde syndrome: Experiences with the use of semiautomatic vWF multimer analysis for diagnosis and TAVI for treatment - case report.

Medicine
2025

Acquired von Willebrand syndrome caused by paravalvular prosthetic mitral regurgitation.

European heart journal
2025

Transcatheter Aortic Valve Replacement Reverses Heyde Syndrome: A Case Report of Severe Aortic Stenosis and Gastrointestinal Bleeding.

Journal of clinical medicine
2025

Successful Management of Acquired von Willebrand Syndrome Associated with Monoclonal Gammopathy of Undetermined Significance After Sotorasib Treatment in a Patient with Non-Small-Cell Lung Carcinoma.

Hematology reports
2025

Loss of von Willebrand factor large multimers in patients undergoing hemodialysis: A single-center, retrospective study.

Thrombosis research
2025

A case of hemichorea-hemiballismus secondary to a subacute hemorrhage in a patient with essential thrombocythemia: a case report.

Journal of medical case reports
2025

Management of Bleeding, Thrombotic and Pregnancy-Related Complications in Women with Myeloproliferative Neoplasms: A Case-Based Review Focusing on Sex-Specific Challenges.

Journal of clinical medicine
2025

Effectiveness of Transcatheter Aortic Valve Replacement on Acquired von Willebrand Syndrome: A Prospective Cohort Study.

JACC. Cardiovascular interventions
2024

Hemorrhagic Stroke in a 24-Year-Old Male With Polycythemia Vera: A Case Report and Literature Review.

Cureus
2024

Acquired Von Willebrand Syndrome as a Presenting Manifestation of Monoclonal IgM-Producing Non-Hodgkin's B-cell Lymphoma.

Cureus
2024

Letter to editor: Impact of extracorporeal membrane oxygenation treatments on acquired von willebrand syndrome in patients with out of-hospital cardiac arrest: a retrospective observational study.

Thrombosis journal
2024

[Acquired von Willebrand syndrome: case report and literature review].

Praxis
2024

Heyde Syndrome Unveiled: A Case Report with Current Literature Review and Molecular Insights.

International journal of molecular sciences
2025

Misleading antigenic von Willebrand factor levels in acquired von Willebrand syndrome secondary to monoclonal gammopathy of undetermined significance.

International journal of hematology
2024

Efficacy and safety of a recombinant von Willebrand factor treatment in acquired von Willebrand syndrome in case of bleeding and surgical procedures.

Haemophilia : the official journal of the World Federation of Hemophilia
2024

Refractory Iron-Deficiency Anemia and Vascular Malformation Concomitant with Aortic Stenosis: Heyde Syndrome.

The American journal of case reports
2024

Risk of bleeding in patients with essential thrombocythemia and extreme thrombocytosis.

Blood advances
2024

A rare case of acquired von Willebrand syndrome type 2B: diagnosis, treatment, and underlying pathophysiology.

Research and practice in thrombosis and haemostasis
2024

Thrombocytopenia and Bleeding in Chronic Kidney Disease: A Case of Acquired Von Willebrand Syndrome.

Cureus
2024

[Evaluation of a semi-automated test for quantification of von Willebrand multimers].

Annales de biologie clinique
2024

The clinical impact of acquired von Willebrand syndrome secondary to Waldenström macroglobulinemia: an underrecognized source of major bleeding events.

Leukemia
2024

Intravenous Immunoglobulin offers temporary improvement in acquired von Willebrand syndrome due to monoclonal gammopathy: A case report.

EJHaem
2024

Successful treatment of acquired von Willebrand syndrome secondary to low-grade CLL with rituximab and venetoclax.

Leukemia &amp; lymphoma
2025

Critical hematological parameters in bleeding during extracorporeal membrane oxygenation support.

Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs
2024

Percutaneous Left Atrial Appendage Closure in a Patient With Acquired Von Willebrand Disease and Atrial Fibrillation.

Cureus
2024

Investigation of von Willebrand factor multimer abnormalities before and after aortic valve replacement using the Hydragel-5 assay.

Thrombosis research
2024

Acquired von Willebrand syndrome during extracorporeal membrane oxygenation support: a comprehensive review of current evidence: communication from the ISTH SSC on perioperative and critical care thrombosis and hemostasis.

Journal of thrombosis and haemostasis : JTH
2024

Mitral regurgitation is associated with similar loss of von Willebrand factor large multimers but lower frequency of anemia compared with aortic stenosis.

Research and practice in thrombosis and haemostasis
2024

Impact of extracorporeal membrane oxygenation treatments on acquired von Willebrand syndrome in patients with out-of-hospital cardiac arrest: a retrospective observational study.

Thrombosis journal
2024

Retrospectively diagnosed autoimmune VWF deficiency in a patient with repeated hemorrhagic events after two common colds.

International journal of hematology
2024

Small intestinal angiodysplasia and hemorrhage associated with acquired von Willebrand syndrome type 2A secondary to multiple myeloma: Reversal after treatment with IRD regimen (a case report and a brief review of the literature).

Thrombosis research
2024

Heyde's syndrome: a challenging case of severe aortic stenosis and gastrointestinal bleeding.

BMJ case reports
2024

A Review About the Assessment of the Bleeding and Thrombosis Risk for Patients With Myeloproliferative Neoplasms Scheduled for Surgery.

Cureus
2024

Acquired von Willebrand Syndrome in a 17-Year-Old With Essential Thrombocythemia: A Case Report With Literature Review.

Cureus
2024

Sustained good response to rituximab in acquired von Willebrand syndrome.

Blood coagulation &amp; fibrinolysis : an international journal in haemostasis and thrombosis
2024

Use of Vonicog Alpha and Acquired von Willebrand Syndrome, a New Approach: A Case Report.

Hamostaseologie
2024

Evidence That Anemia Accelerates AS Progression Via Shear-Induced TGF-β1 Activation: Heyde's Syndrome Comes Full Circle.

JACC. Basic to translational science
2024

Case report: Peri-procedural hydroxyurea helps minimize bleeding in patients with Essential Thrombocythemia associated with acquired von Willebrand syndrome.

Frontiers in oncology
2024

Acquired von Willebrand Syndrome Associated with a Smoldering Multiple Myeloma, Successfully Treated by Daratumumab, Lenalidomide, and Dexamethasone.

Acta haematologica
2024

Acquired von Willebrand syndrome in a patient with monoclonal gammopathy of unknown significance: A case report.

Clinical case reports
2024

Acquired von Willebrand syndrome and post-operative drainage: a comparison of patients with aortic stenosis versus coronary artery disease.

General thoracic and cardiovascular surgery
2024

von Willebrand factor Ristocetin co-factor activity to von Willebrand factor antigen level ratio for diagnosis of acquired von Willebrand syndrome caused by aortic stenosis.

Research and practice in thrombosis and haemostasis
2023

Acquired von Willebrand Syndrome in a Patient Undergoing Extracorporeal Membrane Oxygenation: A Case Report.

Cureus
2024

The left ventricular assist device: a literature review and guidelines for dental care.

General dentistry
2023

Surprise diagnosis of acquired von Willebrand syndrome in a patient previously thought to have type III von Willebrand disease: evaluation and periprocedural management.

BMJ case reports
2023

Influence of Aortic Valve Stenosis and Wall Shear Stress on Platelets Function.

Journal of clinical medicine
2023

The Influence of Hypothyroid Metabolic Status on Blood Coagulation and the Acquired von Willebrand Syndrome.

Journal of clinical medicine
2023

Development of an in-vitro model for extracorporeal blood pumps to study the effects of artificial pulsatility on human blood.

Frontiers in medicine
2024

How I treat von Willebrand disorders in older adults.

Blood
2024

Same Syndrome, Different Causes and Treatment: Path to Diagnosis and Management of Two Interesting Cases of Acquired von Willebrand Syndrome.

Seminars in thrombosis and hemostasis
2023

Effectiveness of aortic valve replacement in Heyde syndrome: a meta-analysis.

European heart journal
2023

Beyond Acquired Von Willebrand Deficiency: Exploring Alternative Mechanisms of Heyde's Syndrome.

Cureus
2023

Shear-induced acquired von Willebrand syndrome: an accomplice of bleeding events in adults on extracorporeal membrane oxygenation support.

Frontiers in cardiovascular medicine
2023

Did angiodysplasia associated with heyde's syndrome disappear spontaneously?: a case report.

Journal of cardiothoracic surgery
2023

Gastrointestinal Bleeding in Patients Supported with Left Ventricular Assist Devices: The Journey from Bridging to Destination.

The American journal of cardiology
2023

Hemostatic disorders associated with extracorporeal membrane oxygenation.

Minerva anestesiologica
2023

Gastrointestinal bleeding in von Willebrand patients: special diagnostic and management considerations.

Expert review of hematology
2023

Laboratory Testing for von Willebrand Disease Using a Composite Rapid 3-Test Chemiluminescence-Based von Willebrand Factor Assay Panel.

Methods in molecular biology (Clifton, N.J.)
2023

Long-term assist device patients admitted to ICU: Tips and pitfalls.

Journal of intensive medicine
2023

The endogenous thrombin potential in patients with left ventricular assist device or heart transplant.

Frontiers in medicine
2023

An unusual cause of iron deficiency anemia in a patient with severe aortic stenosis and heart failure.

Journal of cardiology cases
2023

[Rare manifestations of monoclonal gammopathies: About two clinical cases and literature review].

La Revue de medecine interne
2023

Acquired von willebrand syndrome secondary to monoclonal gammopathy of undetermined significance: long-term remission after treatment with bortezomib.

Journal of thrombosis and thrombolysis
2023

Heyde Syndrome Complicated by Essential Thrombocythemia: A Case Report.

Cureus
2023

Thrombotic thrombocytopenic purpura in caplacizumab era - An individualized approach.

Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
2024

Relapse of Acquired Hemophilia A after COVID-19 Infection.

Journal of Nippon Medical School = Nippon Ika Daigaku zasshi
2023

Acquired Von Willebrand Syndrome following a SARS-CoV2 Infection.

Acta haematologica
2024

The Role of the von Willebrand Factor Collagen-Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36-Year History.

Seminars in thrombosis and hemostasis
2023

Acquired von willebrand syndrome in patients with philadelphia-negative myeloproliferative neoplasm.

Blood research
2023

Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation.

Journal of clinical medicine
2023

Current and future strategies to monitor and manage coagulation in ECMO patients.

Thrombosis journal
2023

The effect of recombinant versus plasma-derived von Willebrand factor on prolonged PFA closure times in ECMO patients with acquired von Willebrand syndrome - an observational study.

Thrombosis journal
2022

Acquired von Willebrand syndrome is common in infants with systemic-to-pulmonary shunts: Retrospective case-series.

Frontiers in pediatrics
2022

Comprehensive Analyses of Coagulation Parameters in Patients with Vascular Anomalies.

Biomolecules
2022

Acquired von Willebrand Syndrome Secondary to Normally Functioning Mechanical Aortic Valve and High-Output Cardiac State.

Journal of cardiovascular development and disease
2022

Successful and safe response to ibrutinib alone in treating relapsed Waldenström macrogobulinemia and related acquired von Willebrand syndrome: an option to consider.

Leukemia &amp; lymphoma
2022

Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH).

Frontiers in pediatrics
2023

Loss of pulsatility with continuous-flow left ventricular assist devices and the significance of the arterial endothelium in von-Willebrand factor production and degradation.

Artificial organs
2022

[Acquired von Willebrand syndrome (AvWS)].

Vnitrni lekarstvi
2022

Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage.

Frontiers in pediatrics
2022

Successful use of lenalidomide to treat refractory acquired von Willebrand disease associated with monoclonal gammopathy.

Annals of hematology
2022

ADAMTS13 inhibition to treat acquired von Willebrand syndrome during mechanical circulatory support device implantation.

Journal of thrombosis and haemostasis : JTH
2022

[Cancer-associated coagulation disorders].

Dermatologie (Heidelberg, Germany)
2022

Menorrhagia as main presentation sign of severe hypothyroidism in a pediatric patient: a case report.

Italian journal of pediatrics
2022

Laboratory Diagnosis of von Willebrand Disease (VWD): Geographical Perspectives.

Seminars in thrombosis and hemostasis
2023

Perioperative diagnosis and impact of acquired von Willebrand syndrome in infants with congenital heart disease.

Blood
2022

Lymphoma-associated acquired von Willebrand syndrome responsive to splenectomy: A case report.

EJHaem
2022

Acquired von Willebrand syndrome: Five cases report and literature review.

Thrombosis research
2022

Acquired von Willebrand syndrome and lymphoid neoplasms: A review of malignancy management, and propositions of practical recommendations.

Haemophilia : the official journal of the World Federation of Hemophilia
2022

Evaluation of different diagnostic tools for detection of acquired von Willebrand syndrome in patients with polycythemia vera or essential thrombocythemia.

Thrombosis research
2022

Concealed by the convenient: acquired von Willebrand syndrome in myeloproliferative neoplasm requires a thorough evaluation.

Annals of hematology
2023

A breath-taking total knee arthroplasty in a patient with acquired von willebrand syndrome: A case report and literature review.

Asian journal of surgery
2022

Successful Chemical Synovectomy in a Patient with Acquired von Willebrand Syndrome with Chronic Synovitis Due to Recurrent Knee Hemarthrosis: A Case Report.

Rheumatology and therapy
2022

A COVID Constellation: A Case of Transverse Myelitis and Acquired von Willebrand Syndrome.

Journal of investigative medicine high impact case reports
2023

Acquired von Willebrand Syndrome in a Patient with Multiple Comorbidities, Including MALT Lymphoma with IgA Monoclonal Gammopathy and Hyperviscosity Syndrome.

Internal medicine (Tokyo, Japan)
2022

Sixth Åland Island Conference on von Willebrand disease.

Haemophilia : the official journal of the World Federation of Hemophilia
2022

The impact of bicuspid aortic valve morphology on von Willebrand factor function in patients with severe aortic stenosis and its change after TAVI.

Clinical research in cardiology : official journal of the German Cardiac Society
2022

Treatment Algorithm for Patients With von Willebrand Syndrome Type 2A and Congenital Heart Disease-A Treatment Algorithm May Reduce Perioperative Blood Loss in Children With Congenital Heart Disease.

Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
2022

Systemic bevacizumab as salvage therapy for persistent severe bleeding and anemia in heyde syndrome following aortic valve replacement.

Journal of thrombosis and thrombolysis
2022

A Review of Autoimmune Acquired von Willebrand Factor Deficiency in Japan.

Seminars in thrombosis and hemostasis
2022

Evaluating Performance of Contemporary and Historical von Willebrand Factor (VWF) Assays in the Laboratory Identification of von Willebrand Disease (VWD): The Australasian Experience.

Seminars in thrombosis and hemostasis
2022

Practical management of the haemorrhagic complications of myeloproliferative neoplasms.

British journal of haematology
2022

Bleeding During Veno-Venous ECMO: Prevention and Treatment.

Frontiers in medicine
2022

Acquired platelet defects are responsible for nonsurgical bleeding in left ventricular assist device recipients.

Artificial organs
2023

More to Learn About Acquired Von Willebrand Syndrome.

ASAIO journal (American Society for Artificial Internal Organs : 1992)
2022

Anticoagulation for Percutaneous Ventricular Assist Device-Supported Cardiogenic Shock: JACC Review Topic of the Week.

Journal of the American College of Cardiology
2022

Heyde's syndrome: a systematic review of case reports.

BMJ open gastroenterology
2022

Acquired bleeding disorders.

Haemophilia : the official journal of the World Federation of Hemophilia
2022

Hyperadhesive von Willebrand Factor Promotes Extracellular Vesicle-Induced Angiogenesis: Implication for LVAD-Induced Bleeding.

JACC. Basic to translational science
2022

Increased cleavage of von Willebrand factor by ADAMTS13 may contribute strongly to acquired von Willebrand syndrome development in patients with essential thrombocythemia.

Journal of thrombosis and haemostasis : JTH
2022

The Intriguing Connections between von Willebrand Factor, ADAMTS13 and Cancer.

Healthcare (Basel, Switzerland)
2022

HemosIL VWF:GPIbR Assay Has a Greater Sensitivity than VWF:RCo Technique to Detect Acquired von Willebrand Syndrome in Myeloproliferative Neoplasms.

Thrombosis and haemostasis
2022

Successful treatment of acquired von Willebrand syndrome associated with monoclonal gammopathy : Breaking a dangerous bond.

Wiener klinische Wochenschrift
2022

Acquired von Willebrand Syndrome and Desmopressin Resistance During Venovenous Extracorporeal Membrane Oxygenation in Patients With COVID-19: A Prospective Observational Study.

Critical care medicine
2022

Impact of the Severity of Acquired von Willebrand Syndrome on the Short-Term Prognosis in Patients with Temporary Mechanical Circulatory Support.

Medicina (Kaunas, Lithuania)
2022

Bioprosthetic Total Artificial Heart in Autoregulated Mode Is Biologically Hemocompatible: Insights for Multimers of von Willebrand Factor.

Arteriosclerosis, thrombosis, and vascular biology
2022

[Relapse of acquired von Willebrand syndrome in a patient non-compliant with Crohn's disease].

Annales de biologie clinique
2022

Distinct Mechanisms of IgM Antibody-Mediated Acquired von Willebrand Syndrome and Successful Treatment with Recombinant von Willebrand Factor in One Patient.

Acta haematologica
2022

Individual Variability in von Willebrand Factor Fragility in Response to Shear Stress: A Possible Clue for Predicting Bleeding Risk.

ASAIO journal (American Society for Artificial Internal Organs : 1992)
2022

Von Willebrand factor and disease: a review for laboratory professionals.

Critical reviews in clinical laboratory sciences
2022

In Vitro Comparison of Recombinant and Plasma-Derived von Willebrand Factor Concentrate for Treatment of Acquired von Willebrand Syndrome in Adult Extracorporeal Membrane Oxygenation Patients.

Anesthesia and analgesia
2021

Role of acquired von Willebrand syndrome in the development of bleeding complications in patients treated with Impella RP devices.

Scientific reports
2022

Acquired von Willebrand syndrome secondary to lymphoproliferative disorders: A case series from two French centers.

Thrombosis research
2023

Acquired von Willebrand syndrome in patients with monoclonal gammopathy of undetermined significance investigated using a mechanistic approach.

Blood transfusion = Trasfusione del sangue
2022

Bortezomib provides favorable efficacy in type 3 acquired von willebrand syndrome related to lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia.

Leukemia &amp; lymphoma
2021

Aortic stenosis and Heyde's syndrome: A comprehensive review.

World journal of clinical cases
2021

Von Willebrand Factor Multimeric Assay in Acquired von Willebrand Disease Diagnosis: A Report of Experience from North Estonia Medical Centre.

Journal of laboratory physicians
2021

Thrombocytapheresis for acquired von Willebrand syndrome in a patient with essential thrombocythemia and recent multivisceral transplantation.

Transfusion
2021

Ibrutinib effect in acquired von Willebrand syndrome secondary to Waldenström macroglobulinemia.

Therapeutic advances in hematology
2021

Subdural Hemorrhage Due to Acquired Von Willebrand Syndrome in a Patient With Polycythemia Vera.

Cureus
2021

A Case of Acquired von Willebrand Syndrome Complicated by Acute Myelomonocytic Leukemia.

Case reports in oncology
2021

A Continuum Model for the Unfolding of von Willebrand Factor.

Annals of biomedical engineering
2021

Acquired von Willebrand Syndrome.

Hematology/oncology clinics of North America
2022

Perspectives on Heyde's Syndrome and Calcific Aortic Valve Disease.

Current problems in cardiology
2021

Acquired von Willebrand syndrome in myeloproliferative neoplasms with extreme thrombocytosis.

Hematological oncology
2021

Effects of Thyroid Function on Hemostasis, Coagulation, and Fibrinolysis: A Mendelian Randomization Study.

Thyroid : official journal of the American Thyroid Association
2021

[Pembrolizumab-induced hypothyreosis and subcutaneous bleeding].

Vnitrni lekarstvi
2021

Rupture of splenic artery aneurysm in a man with polycythemia vera and acquired von Willebrand syndrome.

BMJ case reports
2021

New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome.

HemaSphere
2022

Optimal Tests to Minimise Bleeding and Ischaemic Complications in Patients on Extracorporeal Membrane Oxygenation.

Thrombosis and haemostasis
2021

Daratumumab as a novel treatment for refractory acquired von Willebrand syndrome associated with monoclonal gammopathy.

Haemophilia : the official journal of the World Federation of Hemophilia
2021

Bleeding Diathesis in Multiple Myeloma: A Rare Presentation of a Dreadful Emergency With Management Nightmare.

Cureus
2022

What factors determine the pregnancy outcome in patients with essential thrombocythemia?

The journal of maternal-fetal &amp; neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
2021

Acquired von Willebrand syndrome in monoclonal gammopathy - A scoping review on hemostatic management.

Research and practice in thrombosis and haemostasis
2021

Hypercoagulopathy, acquired coagulation disorders and anticoagulation before, during and after extracorporeal membrane oxygenation in COVID-19: a case series.

Perfusion
2021

Differential diagnostic and treatment difficulties in a patient with acquired von Willebrand syndrome.

Hematology (Amsterdam, Netherlands)
2021

von Willebrand disease: what does the future hold?

Blood
2021

Heyde Syndrome: An Unusual Cause of Gastrointestinal Bleeding.

Journal of investigative medicine high impact case reports
2021

Use of recombinant Von Willebrand factor during transcatheter aortic valve replacement in a patient with acquired von Willebrand syndrome.

Transfusion medicine (Oxford, England)
2022

Successful Perioperative Management of Orthotopic Cardiac Transplantation in a Pediatric Patient With Concurrent Congenital von Willebrand Disease and Acquired von Willebrand Syndrome Using Recombinant von Willebrand Factor.

Journal of cardiothoracic and vascular anesthesia
2021

Anticoagulation and bone marrow biopsy: is it safe to proceed?

Hematology (Amsterdam, Netherlands)
2021

Device-Induced Hemostatic Disorders in Mechanically Assisted Circulation.

Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis
2021

To Treat or Not? Remission Induction of Acquired von Willebrand Syndrome Secondary to Chronic Lymphocytic Leukemia: A Case Report.

Clinical lymphoma, myeloma &amp; leukemia
2021

Acquired von Willebrand syndrome in association with multiple myeloma: remission after stem cell transplant.

BMJ case reports
2021

Etiology and Management of Bleeding during ECMO in a COVID-19 Patient.

Journal of atherosclerosis and thrombosis
2020

Contact activation and acquired von Willebrand syndrome during neonatal extracorporeal circulation.

Journal of thrombosis and haemostasis : JTH
2020

Asymptomatic Severe Acquired von Willebrand's Syndrome in Association With a Glioblastoma Multiforme: A Case Report.

Cureus
2021

Acquired von Willebrand syndrome and factor VIII in patients with moderate to severe mitral regurgitation undergoing transcatheter mitral valve repair.

Clinical cardiology
2021

Acquired von Willebrand syndrome in ECMO patients: A 3-year cohort study.

Blood cells, molecules &amp; diseases
2021

Dynamics of Blood Flows in Aortic Stenosis: Mild, Moderate, and Severe.

ASAIO journal (American Society for Artificial Internal Organs : 1992)
2021

Management of a COVID-19 Patient during ECMO: Paying Attention to Acquired von Willebrand Syndrome.

Journal of atherosclerosis and thrombosis
2020

Pathologic Shear and Elongation Rates Do Not Cause Cleavage of Von Willebrand Factor by ADAMTS13 in a Purified System.

Cellular and molecular bioengineering
2020

Longevity in a Patient With Hypertrophic Cardiomyopathy, Anomalous Coronary Artery, and Gastrointestinal Bleeding.

Cureus
2020

Management of a Pediatric Patient With a Left Ventricular Assist Device and Symptomatic Acquired von Willebrand Syndrome Presenting for Orthotopic Heart Transplant.

Seminars in cardiothoracic and vascular anesthesia
2020

[Acquired von Willebrand syndrome].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2020

[Acquired coagulation disorders].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2021

Von Willebrand Factor Concentrate Administration for Acquired Von Willebrand Syndrome- Related Bleeding During Adult Extracorporeal Membrane Oxygenation.

Journal of cardiothoracic and vascular anesthesia
Ver todos os 347 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Heyde Syndrome: A Literature Review.
    Health science reports· 2026· PMID 41623372mais citado
  2. Thrombotic and Bleeding Complications in Myeloproliferative Neoplasms: An Integrated Clinical Perspective.
    Seminars in thrombosis and hemostasis· 2026· PMID 41856500mais citado
  3. Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
    Journal of thrombosis and haemostasis : JTH· 2026· PMID 41850491mais citado
  4. Efficacy of venetoclax-obinutuzumab treatment in achieving remission of acquired von Willebrand syndrome in chronic lymphocytic leukemia.
    Annals of hematology· 2026· PMID 41831041mais citado
  5. Heyde syndrome secondary to hypertrophic obstructive cardiomyopathy: a case report.
    The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology· 2026· PMID 41801542mais citado
  6. Optimizing treatment for Waldenström macroglobulinemia-associated acquired von Willebrand syndrome: a case report and literature review.
    Front Oncol· 2026· PMID 41982238recente
  7. Acquired von Willebrand Syndrome and Hemolysis in High-Shear States: Time for Cardio-Hematology Collaboration.
    JACC Asia· 2026· PMID 41949248recente
  8. Ischemic Stroke in a Case of Myeloproliferative Neoplasm and Acquired Von Willebrand Syndrome.
    Mayo Clin Proc· 2026· PMID 41903929recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:99147(Orphanet)
  2. MONDO:0020460(MONDO)
  3. GARD:5573(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q50349812(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Doença de von Willebrand adquirida
Compêndio · Raras BR

Doença de von Willebrand adquirida

ORPHA:99147 · MONDO:0020460
🇧🇷 Brasil SUS
CEAF
1AFator VIII de von Willebrand
Geral
Prevalência
<1 / 1 000 000
Casos
300 casos conhecidos
Herança
Not applicable
CID-10
D68.4 · Deficiência adquirida de fator de coagulação
CID-11
Ensaios
2 ativos
Medicamentos
1 registrados
Início
Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0272362
EuropePMC
Wikidata
Papers 10a
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