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.
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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.
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Sinais e sintomas
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Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
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Publicações mais relevantes
Heyde Syndrome: A Literature Review.
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.
Thrombotic and Bleeding Complications in Myeloproliferative Neoplasms: An Integrated Clinical Perspective.
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.
Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
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.
Efficacy of venetoclax-obinutuzumab treatment in achieving remission of acquired von Willebrand syndrome in chronic lymphocytic leukemia.
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.
Heyde syndrome secondary to hypertrophic obstructive cardiomyopathy: a case report.
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
Optimizing treatment for Waldenström macroglobulinemia-associated acquired von Willebrand syndrome: a case report and literature review.
Acquired von Willebrand Syndrome and Hemolysis in High-Shear States: Time for Cardio-Hematology Collaboration.
Ischemic Stroke in a Case of Myeloproliferative Neoplasm and Acquired Von Willebrand Syndrome.
Thrombotic and Bleeding Complications in Myeloproliferative Neoplasms: An Integrated Clinical Perspective.
Efficacy of venetoclax-obinutuzumab treatment in achieving remission of acquired von Willebrand syndrome in chronic lymphocytic leukemia.
📚 EuropePMC347 artigos no totalmostrando 198
Thrombotic and Bleeding Complications in Myeloproliferative Neoplasms: An Integrated Clinical Perspective.
Seminars in thrombosis and hemostasisMonoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
Journal of thrombosis and haemostasis : JTHEfficacy of venetoclax-obinutuzumab treatment in achieving remission of acquired von Willebrand syndrome in chronic lymphocytic leukemia.
Annals of hematologyHeyde syndrome secondary to hypertrophic obstructive cardiomyopathy: a case report.
The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiologyvon Willebrand factor in hemodialysis patients. Does acquired von Willebrand syndrome occur?
Thrombosis researchvon Willebrand factor dynamics in trauma managed with extracorporeal life support.
Journal of thrombosis and haemostasis : JTHHeyde Syndrome: A Literature Review.
Health science reportsBudd-Chiari Syndrome Secondary to Essential Thrombocythaemia Complicated by Acquired Von Willebrand Disease and Mimicking Hepatic Malignancy: A Case Report.
CureusEffect 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)Acquired von Willebrand Syndrome: A Comprehensive Review and a Nordic Perspective.
Seminars in thrombosis and hemostasis[Diffuse large B-cell lymphoma with acquired von Willebrand syndrome: a case report and literature review].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiAnticoagulation and hemostasis in the extracorporeal membrane oxygenation patient.
Current opinion in anaesthesiologyHeyde's Syndrome: Presentation and Successful Treatment With Transcatheter Aortic Valve Implantation (TAVI).
CureusHeterogeneous Hemostatic Disorder Associated with IgA Monoclonal Gammopathy: A Case Series.
Endocrine, metabolic & immune disorders drug targetsConcomitant Acquired Hemophilia A and Acquired Von Willebrand Syndrome from Distinctive Autoantibodies: Case Report.
Hematology reportsAcquired von Willebrand syndrome in children.
Research and practice in thrombosis and haemostasisManaging High Risk Pregnancy in Single Ventricle Physiology with Acquired von Willebrand Disease: A Case Report.
Reports (MDPI)Stroke in Mechanical Circulatory Supported Cardiogenic Shock.
Thrombosis and haemostasisAcquired 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 haemostasisSuccessful Treatment of Acquired von Willebrand Syndrome in a Rare Case of Nodal Marginal Zone Lymphoma With Rituximab Therapy: A Case Report.
CureusComplications at Diagnosis of Pediatric Chronic Myeloid Leukemia in Chronic Phase.
Pediatric blood & cancerThrombocytosis 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 haemostasisVON WILLEBRAND FACTOR IN ECMO: A DYNAMIC MODULATOR OF HEMORRHAGE AND THROMBOSIS.
Shock (Augusta, Ga.)Bleeding Complications in JAK2-Variant Essential Thrombocythemia: A Revisit in 2025.
EJHaemAcquired von Willebrand syndrome secondary to monoclonal gammopathy: a single-center case series.
Therapeutic advances in hematologyVon Willebrand factor is a multifaceted player in hemostasis requiring a diverse array of analytical and diagnostic approaches.
Expert review of hematologyWilms 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/oncologyBleeding due to a Broken Heart Valve: Heyde's Syndrome Secondary to Severe Mitral Regurgitation.
Gastro hep advancesPre-procedural abnormal von Willebrand factor function predicts clinical outcomes after Transcatheter Aortic Valve Implantation: a prospective cohort study.
Frontiers in cardiovascular medicineManagement of COVID-19 associated coagulopathy in critically ill patients and the risk of acquired von willebrand syndrome.
Scientific reportsPerioperative management of transcatheter aortic valve implantation in acquired von Willebrand syndrome secondary to monoclonal gammopathy: a case report.
European heart journal. Case reportsHeyde syndrome: Experiences with the use of semiautomatic vWF multimer analysis for diagnosis and TAVI for treatment - case report.
MedicineAcquired von Willebrand syndrome caused by paravalvular prosthetic mitral regurgitation.
European heart journalTranscatheter Aortic Valve Replacement Reverses Heyde Syndrome: A Case Report of Severe Aortic Stenosis and Gastrointestinal Bleeding.
Journal of clinical medicineSuccessful 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 reportsLoss of von Willebrand factor large multimers in patients undergoing hemodialysis: A single-center, retrospective study.
Thrombosis researchA case of hemichorea-hemiballismus secondary to a subacute hemorrhage in a patient with essential thrombocythemia: a case report.
Journal of medical case reportsManagement of Bleeding, Thrombotic and Pregnancy-Related Complications in Women with Myeloproliferative Neoplasms: A Case-Based Review Focusing on Sex-Specific Challenges.
Journal of clinical medicineEffectiveness of Transcatheter Aortic Valve Replacement on Acquired von Willebrand Syndrome: A Prospective Cohort Study.
JACC. Cardiovascular interventionsHemorrhagic Stroke in a 24-Year-Old Male With Polycythemia Vera: A Case Report and Literature Review.
CureusAcquired Von Willebrand Syndrome as a Presenting Manifestation of Monoclonal IgM-Producing Non-Hodgkin's B-cell Lymphoma.
CureusLetter 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[Acquired von Willebrand syndrome: case report and literature review].
PraxisHeyde Syndrome Unveiled: A Case Report with Current Literature Review and Molecular Insights.
International journal of molecular sciencesMisleading antigenic von Willebrand factor levels in acquired von Willebrand syndrome secondary to monoclonal gammopathy of undetermined significance.
International journal of hematologyEfficacy 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 HemophiliaRefractory Iron-Deficiency Anemia and Vascular Malformation Concomitant with Aortic Stenosis: Heyde Syndrome.
The American journal of case reportsRisk of bleeding in patients with essential thrombocythemia and extreme thrombocytosis.
Blood advancesA rare case of acquired von Willebrand syndrome type 2B: diagnosis, treatment, and underlying pathophysiology.
Research and practice in thrombosis and haemostasisThrombocytopenia and Bleeding in Chronic Kidney Disease: A Case of Acquired Von Willebrand Syndrome.
Cureus[Evaluation of a semi-automated test for quantification of von Willebrand multimers].
Annales de biologie cliniqueThe clinical impact of acquired von Willebrand syndrome secondary to Waldenström macroglobulinemia: an underrecognized source of major bleeding events.
LeukemiaIntravenous Immunoglobulin offers temporary improvement in acquired von Willebrand syndrome due to monoclonal gammopathy: A case report.
EJHaemSuccessful treatment of acquired von Willebrand syndrome secondary to low-grade CLL with rituximab and venetoclax.
Leukemia & lymphomaCritical hematological parameters in bleeding during extracorporeal membrane oxygenation support.
Journal of artificial organs : the official journal of the Japanese Society for Artificial OrgansPercutaneous Left Atrial Appendage Closure in a Patient With Acquired Von Willebrand Disease and Atrial Fibrillation.
CureusInvestigation of von Willebrand factor multimer abnormalities before and after aortic valve replacement using the Hydragel-5 assay.
Thrombosis researchAcquired 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 : JTHMitral 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 haemostasisImpact of extracorporeal membrane oxygenation treatments on acquired von Willebrand syndrome in patients with out-of-hospital cardiac arrest: a retrospective observational study.
Thrombosis journalRetrospectively diagnosed autoimmune VWF deficiency in a patient with repeated hemorrhagic events after two common colds.
International journal of hematologySmall 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 researchHeyde's syndrome: a challenging case of severe aortic stenosis and gastrointestinal bleeding.
BMJ case reportsA Review About the Assessment of the Bleeding and Thrombosis Risk for Patients With Myeloproliferative Neoplasms Scheduled for Surgery.
CureusAcquired von Willebrand Syndrome in a 17-Year-Old With Essential Thrombocythemia: A Case Report With Literature Review.
CureusSustained good response to rituximab in acquired von Willebrand syndrome.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisUse of Vonicog Alpha and Acquired von Willebrand Syndrome, a New Approach: A Case Report.
HamostaseologieEvidence That Anemia Accelerates AS Progression Via Shear-Induced TGF-β1 Activation: Heyde's Syndrome Comes Full Circle.
JACC. Basic to translational scienceCase report: Peri-procedural hydroxyurea helps minimize bleeding in patients with Essential Thrombocythemia associated with acquired von Willebrand syndrome.
Frontiers in oncologyAcquired von Willebrand Syndrome Associated with a Smoldering Multiple Myeloma, Successfully Treated by Daratumumab, Lenalidomide, and Dexamethasone.
Acta haematologicaAcquired von Willebrand syndrome in a patient with monoclonal gammopathy of unknown significance: A case report.
Clinical case reportsAcquired von Willebrand syndrome and post-operative drainage: a comparison of patients with aortic stenosis versus coronary artery disease.
General thoracic and cardiovascular surgeryvon 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 haemostasisAcquired von Willebrand Syndrome in a Patient Undergoing Extracorporeal Membrane Oxygenation: A Case Report.
CureusThe left ventricular assist device: a literature review and guidelines for dental care.
General dentistrySurprise diagnosis of acquired von Willebrand syndrome in a patient previously thought to have type III von Willebrand disease: evaluation and periprocedural management.
BMJ case reportsInfluence of Aortic Valve Stenosis and Wall Shear Stress on Platelets Function.
Journal of clinical medicineThe Influence of Hypothyroid Metabolic Status on Blood Coagulation and the Acquired von Willebrand Syndrome.
Journal of clinical medicineDevelopment of an in-vitro model for extracorporeal blood pumps to study the effects of artificial pulsatility on human blood.
Frontiers in medicineHow I treat von Willebrand disorders in older adults.
BloodSame Syndrome, Different Causes and Treatment: Path to Diagnosis and Management of Two Interesting Cases of Acquired von Willebrand Syndrome.
Seminars in thrombosis and hemostasisEffectiveness of aortic valve replacement in Heyde syndrome: a meta-analysis.
European heart journalBeyond Acquired Von Willebrand Deficiency: Exploring Alternative Mechanisms of Heyde's Syndrome.
CureusShear-induced acquired von Willebrand syndrome: an accomplice of bleeding events in adults on extracorporeal membrane oxygenation support.
Frontiers in cardiovascular medicineDid angiodysplasia associated with heyde's syndrome disappear spontaneously?: a case report.
Journal of cardiothoracic surgeryGastrointestinal Bleeding in Patients Supported with Left Ventricular Assist Devices: The Journey from Bridging to Destination.
The American journal of cardiologyHemostatic disorders associated with extracorporeal membrane oxygenation.
Minerva anestesiologicaGastrointestinal bleeding in von Willebrand patients: special diagnostic and management considerations.
Expert review of hematologyLaboratory 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.)Long-term assist device patients admitted to ICU: Tips and pitfalls.
Journal of intensive medicineThe endogenous thrombin potential in patients with left ventricular assist device or heart transplant.
Frontiers in medicineAn unusual cause of iron deficiency anemia in a patient with severe aortic stenosis and heart failure.
Journal of cardiology cases[Rare manifestations of monoclonal gammopathies: About two clinical cases and literature review].
La Revue de medecine interneAcquired von willebrand syndrome secondary to monoclonal gammopathy of undetermined significance: long-term remission after treatment with bortezomib.
Journal of thrombosis and thrombolysisHeyde Syndrome Complicated by Essential Thrombocythemia: A Case Report.
CureusThrombotic 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 HaemapheresisRelapse of Acquired Hemophilia A after COVID-19 Infection.
Journal of Nippon Medical School = Nippon Ika Daigaku zasshiAcquired Von Willebrand Syndrome following a SARS-CoV2 Infection.
Acta haematologicaThe 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 hemostasisAcquired von willebrand syndrome in patients with philadelphia-negative myeloproliferative neoplasm.
Blood researchMild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation.
Journal of clinical medicineCurrent and future strategies to monitor and manage coagulation in ECMO patients.
Thrombosis journalThe 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 journalAcquired von Willebrand syndrome is common in infants with systemic-to-pulmonary shunts: Retrospective case-series.
Frontiers in pediatricsComprehensive Analyses of Coagulation Parameters in Patients with Vascular Anomalies.
BiomoleculesAcquired von Willebrand Syndrome Secondary to Normally Functioning Mechanical Aortic Valve and High-Output Cardiac State.
Journal of cardiovascular development and diseaseSuccessful and safe response to ibrutinib alone in treating relapsed Waldenström macrogobulinemia and related acquired von Willebrand syndrome: an option to consider.
Leukemia & lymphomaAcquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH).
Frontiers in pediatricsLoss 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[Acquired von Willebrand syndrome (AvWS)].
Vnitrni lekarstviCase report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage.
Frontiers in pediatricsSuccessful use of lenalidomide to treat refractory acquired von Willebrand disease associated with monoclonal gammopathy.
Annals of hematologyADAMTS13 inhibition to treat acquired von Willebrand syndrome during mechanical circulatory support device implantation.
Journal of thrombosis and haemostasis : JTH[Cancer-associated coagulation disorders].
Dermatologie (Heidelberg, Germany)Menorrhagia as main presentation sign of severe hypothyroidism in a pediatric patient: a case report.
Italian journal of pediatricsLaboratory Diagnosis of von Willebrand Disease (VWD): Geographical Perspectives.
Seminars in thrombosis and hemostasisPerioperative diagnosis and impact of acquired von Willebrand syndrome in infants with congenital heart disease.
BloodLymphoma-associated acquired von Willebrand syndrome responsive to splenectomy: A case report.
EJHaemAcquired von Willebrand syndrome: Five cases report and literature review.
Thrombosis researchAcquired 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 HemophiliaEvaluation of different diagnostic tools for detection of acquired von Willebrand syndrome in patients with polycythemia vera or essential thrombocythemia.
Thrombosis researchConcealed by the convenient: acquired von Willebrand syndrome in myeloproliferative neoplasm requires a thorough evaluation.
Annals of hematologyA breath-taking total knee arthroplasty in a patient with acquired von willebrand syndrome: A case report and literature review.
Asian journal of surgerySuccessful Chemical Synovectomy in a Patient with Acquired von Willebrand Syndrome with Chronic Synovitis Due to Recurrent Knee Hemarthrosis: A Case Report.
Rheumatology and therapyA COVID Constellation: A Case of Transverse Myelitis and Acquired von Willebrand Syndrome.
Journal of investigative medicine high impact case reportsAcquired von Willebrand Syndrome in a Patient with Multiple Comorbidities, Including MALT Lymphoma with IgA Monoclonal Gammopathy and Hyperviscosity Syndrome.
Internal medicine (Tokyo, Japan)Sixth Åland Island Conference on von Willebrand disease.
Haemophilia : the official journal of the World Federation of HemophiliaThe 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 SocietyTreatment 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 SocietiesSystemic bevacizumab as salvage therapy for persistent severe bleeding and anemia in heyde syndrome following aortic valve replacement.
Journal of thrombosis and thrombolysisA Review of Autoimmune Acquired von Willebrand Factor Deficiency in Japan.
Seminars in thrombosis and hemostasisEvaluating 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 hemostasisPractical management of the haemorrhagic complications of myeloproliferative neoplasms.
British journal of haematologyBleeding During Veno-Venous ECMO: Prevention and Treatment.
Frontiers in medicineAcquired platelet defects are responsible for nonsurgical bleeding in left ventricular assist device recipients.
Artificial organsMore to Learn About Acquired Von Willebrand Syndrome.
ASAIO journal (American Society for Artificial Internal Organs : 1992)Anticoagulation for Percutaneous Ventricular Assist Device-Supported Cardiogenic Shock: JACC Review Topic of the Week.
Journal of the American College of CardiologyHeyde's syndrome: a systematic review of case reports.
BMJ open gastroenterologyAcquired bleeding disorders.
Haemophilia : the official journal of the World Federation of HemophiliaHyperadhesive von Willebrand Factor Promotes Extracellular Vesicle-Induced Angiogenesis: Implication for LVAD-Induced Bleeding.
JACC. Basic to translational scienceIncreased 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 : JTHThe Intriguing Connections between von Willebrand Factor, ADAMTS13 and Cancer.
Healthcare (Basel, Switzerland)HemosIL VWF:GPIbR Assay Has a Greater Sensitivity than VWF:RCo Technique to Detect Acquired von Willebrand Syndrome in Myeloproliferative Neoplasms.
Thrombosis and haemostasisSuccessful treatment of acquired von Willebrand syndrome associated with monoclonal gammopathy : Breaking a dangerous bond.
Wiener klinische WochenschriftAcquired von Willebrand Syndrome and Desmopressin Resistance During Venovenous Extracorporeal Membrane Oxygenation in Patients With COVID-19: A Prospective Observational Study.
Critical care medicineImpact of the Severity of Acquired von Willebrand Syndrome on the Short-Term Prognosis in Patients with Temporary Mechanical Circulatory Support.
Medicina (Kaunas, Lithuania)Bioprosthetic Total Artificial Heart in Autoregulated Mode Is Biologically Hemocompatible: Insights for Multimers of von Willebrand Factor.
Arteriosclerosis, thrombosis, and vascular biology[Relapse of acquired von Willebrand syndrome in a patient non-compliant with Crohn's disease].
Annales de biologie cliniqueDistinct Mechanisms of IgM Antibody-Mediated Acquired von Willebrand Syndrome and Successful Treatment with Recombinant von Willebrand Factor in One Patient.
Acta haematologicaIndividual 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)Von Willebrand factor and disease: a review for laboratory professionals.
Critical reviews in clinical laboratory sciencesIn 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 analgesiaRole of acquired von Willebrand syndrome in the development of bleeding complications in patients treated with Impella RP devices.
Scientific reportsAcquired von Willebrand syndrome secondary to lymphoproliferative disorders: A case series from two French centers.
Thrombosis researchAcquired von Willebrand syndrome in patients with monoclonal gammopathy of undetermined significance investigated using a mechanistic approach.
Blood transfusion = Trasfusione del sangueBortezomib provides favorable efficacy in type 3 acquired von willebrand syndrome related to lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia.
Leukemia & lymphomaAortic stenosis and Heyde's syndrome: A comprehensive review.
World journal of clinical casesVon Willebrand Factor Multimeric Assay in Acquired von Willebrand Disease Diagnosis: A Report of Experience from North Estonia Medical Centre.
Journal of laboratory physiciansThrombocytapheresis for acquired von Willebrand syndrome in a patient with essential thrombocythemia and recent multivisceral transplantation.
TransfusionIbrutinib effect in acquired von Willebrand syndrome secondary to Waldenström macroglobulinemia.
Therapeutic advances in hematologySubdural Hemorrhage Due to Acquired Von Willebrand Syndrome in a Patient With Polycythemia Vera.
CureusA Case of Acquired von Willebrand Syndrome Complicated by Acute Myelomonocytic Leukemia.
Case reports in oncologyA Continuum Model for the Unfolding of von Willebrand Factor.
Annals of biomedical engineeringAcquired von Willebrand Syndrome.
Hematology/oncology clinics of North AmericaPerspectives on Heyde's Syndrome and Calcific Aortic Valve Disease.
Current problems in cardiologyAcquired von Willebrand syndrome in myeloproliferative neoplasms with extreme thrombocytosis.
Hematological oncologyEffects of Thyroid Function on Hemostasis, Coagulation, and Fibrinolysis: A Mendelian Randomization Study.
Thyroid : official journal of the American Thyroid Association[Pembrolizumab-induced hypothyreosis and subcutaneous bleeding].
Vnitrni lekarstviRupture of splenic artery aneurysm in a man with polycythemia vera and acquired von Willebrand syndrome.
BMJ case reportsNew Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome.
HemaSphereOptimal Tests to Minimise Bleeding and Ischaemic Complications in Patients on Extracorporeal Membrane Oxygenation.
Thrombosis and haemostasisDaratumumab as a novel treatment for refractory acquired von Willebrand syndrome associated with monoclonal gammopathy.
Haemophilia : the official journal of the World Federation of HemophiliaBleeding Diathesis in Multiple Myeloma: A Rare Presentation of a Dreadful Emergency With Management Nightmare.
CureusWhat factors determine the pregnancy outcome in patients with essential thrombocythemia?
The journal of maternal-fetal & 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 ObstetriciansAcquired von Willebrand syndrome in monoclonal gammopathy - A scoping review on hemostatic management.
Research and practice in thrombosis and haemostasisHypercoagulopathy, acquired coagulation disorders and anticoagulation before, during and after extracorporeal membrane oxygenation in COVID-19: a case series.
PerfusionDifferential diagnostic and treatment difficulties in a patient with acquired von Willebrand syndrome.
Hematology (Amsterdam, Netherlands)von Willebrand disease: what does the future hold?
BloodHeyde Syndrome: An Unusual Cause of Gastrointestinal Bleeding.
Journal of investigative medicine high impact case reportsUse of recombinant Von Willebrand factor during transcatheter aortic valve replacement in a patient with acquired von Willebrand syndrome.
Transfusion medicine (Oxford, England)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 anesthesiaAnticoagulation and bone marrow biopsy: is it safe to proceed?
Hematology (Amsterdam, Netherlands)Device-Induced Hemostatic Disorders in Mechanically Assisted Circulation.
Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisTo Treat or Not? Remission Induction of Acquired von Willebrand Syndrome Secondary to Chronic Lymphocytic Leukemia: A Case Report.
Clinical lymphoma, myeloma & leukemiaAcquired von Willebrand syndrome in association with multiple myeloma: remission after stem cell transplant.
BMJ case reportsEtiology and Management of Bleeding during ECMO in a COVID-19 Patient.
Journal of atherosclerosis and thrombosisContact activation and acquired von Willebrand syndrome during neonatal extracorporeal circulation.
Journal of thrombosis and haemostasis : JTHAsymptomatic Severe Acquired von Willebrand's Syndrome in Association With a Glioblastoma Multiforme: A Case Report.
CureusAcquired von Willebrand syndrome and factor VIII in patients with moderate to severe mitral regurgitation undergoing transcatheter mitral valve repair.
Clinical cardiologyAcquired von Willebrand syndrome in ECMO patients: A 3-year cohort study.
Blood cells, molecules & diseasesDynamics of Blood Flows in Aortic Stenosis: Mild, Moderate, and Severe.
ASAIO journal (American Society for Artificial Internal Organs : 1992)Management of a COVID-19 Patient during ECMO: Paying Attention to Acquired von Willebrand Syndrome.
Journal of atherosclerosis and thrombosisPathologic Shear and Elongation Rates Do Not Cause Cleavage of Von Willebrand Factor by ADAMTS13 in a Purified System.
Cellular and molecular bioengineeringLongevity in a Patient With Hypertrophic Cardiomyopathy, Anomalous Coronary Artery, and Gastrointestinal Bleeding.
CureusManagement 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[Acquired von Willebrand syndrome].
[Rinsho ketsueki] The Japanese journal of clinical hematology[Acquired coagulation disorders].
[Rinsho ketsueki] The Japanese journal of clinical hematologyVon Willebrand Factor Concentrate Administration for Acquired Von Willebrand Syndrome- Related Bleeding During Adult Extracorporeal Membrane Oxygenation.
Journal of cardiothoracic and vascular anesthesiaAssociações
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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.
- Heyde Syndrome: A Literature Review.
- Thrombotic and Bleeding Complications in Myeloproliferative Neoplasms: An Integrated Clinical Perspective.
- Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
- Efficacy of venetoclax-obinutuzumab treatment in achieving remission of acquired von Willebrand syndrome in chronic lymphocytic leukemia.
- 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
- Optimizing treatment for Waldenström macroglobulinemia-associated acquired von Willebrand syndrome: a case report and literature review.
- Acquired von Willebrand Syndrome and Hemolysis in High-Shear States: Time for Cardio-Hematology Collaboration.
- Ischemic Stroke in a Case of Myeloproliferative Neoplasm and Acquired Von Willebrand Syndrome.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:99147(Orphanet)
- MONDO:0020460(MONDO)
- GARD:5573(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
