A Doença de von Willebrand tipo 3 (DVW tipo 3) é a forma mais grave dessa doença. Ela é caracterizada por um problema que causa sangramentos, ligado à falta total ou quase total do fator de von Willebrand (FVW) no sangue (plasma) e nas células do corpo. Isso também leva a uma falta muito grande do fator VIII (FVIII) no sangue.
Introdução
O que você precisa saber de cara
A Doença de von Willebrand tipo 3 (DVW tipo 3) é a forma mais grave dessa doença. Ela é caracterizada por um problema que causa sangramentos, ligado à falta total ou quase total do fator de von Willebrand (FVW) no sangue (plasma) e nas células do corpo. Isso também leva a uma falta muito grande do fator VIII (FVIII) no sangue.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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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
+ 7 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 13 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Autosomal recessive.
Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma
SecretedSecreted, extracellular space, extracellular matrix
von Willebrand disease 1
A common hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 1 is characterized by partial quantitative deficiency of circulating von Willebrand factor, that is otherwise structurally and functionally normal. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma.
Medicamentos aprovados (FDA)
1 medicamento encontrado nos registros da FDA americana.
Variantes genéticas (ClinVar)
770 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 311 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
23 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Doença de Von Willebrand, tipo 3
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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
Ensaios em destaque
🟢 Recrutando agora
6 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
7 ensaios clínicos encontrados, 6 ativos.
Publicações mais relevantes
A phase 1, open-label study to assess the pharmacokinetics, safety, and tolerability of a single intravenous injection of efanesoctocog alfa in adults with type 2N or type 3 von Willebrand disease.
Efanesoctocog alfa is a high-sustained factor (F)VIII replacement therapy for hemophilia A. The D'D3 domain of efanesoctocog alfa overcomes the von Willebrand factor-imposed half-life ceiling and may allow correction of reduced FVIII levels experienced by people with type 2N or type 3 von Willebrand disease (VWD). This study aimed to assess the pharmacokinetics, safety, and tolerability of efanesoctocog alfa in type 2N or type 3 VWD. This was a phase 1, open-label study of efanesoctocog alfa in adults with hereditary type 2N or type 3 VWD (NCT04770935). Participants received a single intravenous dose of efanesoctocog alfa (25 IU/kg). The primary end point was pharmacokinetic parameters as determined by noncompartmental analysis of efanesoctocog alfa FVIII activity using one-stage assay and capture chromogenic assay, the latter of which is more relevant in assessing FVIII levels from efanesoctocog alfa among people with VWD. Secondary end points included adverse events and FVIII inhibitor development. Six participants were assessed (type 2N, n = 2; type 3, n = 4). One dose of efanesoctocog alfa of 25 IU/kg maintained FVIII activity levels of >1 IU/dL up to 10 days postdose. Mean baseline-corrected FVIII activity levels were maintained at >40 and >10 IU/dL up to 1 day and 4 days postdose, respectively, per capture chromogenic assay. Mean (standard deviation) t1/2z was 49.0 (10.2) hours. Efanesoctocog alfa was well tolerated. FVIII inhibitors or antidrug antibodies were not detected. Efanesoctocog alfa is well-tolerated and maintains high FVIII activity levels for a prolonged period in patients with type 2N or type 3 VWD.
Management of gastrointestinal bleeding in a patient with type 3 von Willebrand disease and inhibitors to von Willebrand factor: a case report.
von Willebrand disease (VWD) is a rare bleeding disorder caused by deficiency of von Willebrand factor (VWF). Development of inhibiting alloantibodies to VWF in VWD after exposure to factor concentrates is very rare. We present a 56-year-old male patient with type 3 VWD and history of inhibitors to VWF who was hospitalized due to severe bleeding from the rectal angiodysplasia, which happened 6 months after radical irradiation therapy of the prostate adenocarcinoma. He was treated locally with argon plasma coagulation and application of peptide hydrogel, which resulted in progression of lesions to rectal ulcer covering two-thirds of the rectal mucosa. In addition to almost daily transfusions of red blood cells, he received recombinant activated factor VIIa (rFVIIa), tranexamic acid, FXIII concentrate and activated prothrombin complex concentrate (APCC). Infusion of VWF/FVIII concentrate resulted in reappearance of FVIII inhibitors. Ultimately, the bleeding stopped after introduction of off-label emicizumab, platelet transfusions and local application of the platelet-rich fibrin (PRF).
Successful desensitization with FVIII/Von Willebrand Factor concentrate in Type III Von Willebrand Disease.
Von Willebrand Disease (VWD) Type 3 is a rare and severe bleeding disorder characterized by an almost complete deficiency of Von Willebrand Factor (VWF). Plasma-derived Factor VIII (FVIII)/VWF concentrates are used both on demand and for prophylactic treatment. However, allergic reactions to these products pose significant challenges in clinical management. A 40-year-old female patient with VWD Type 3 presented to our clinic with symptoms including jaw numbness and tightness, chills, fatigue, nausea, and dyspnea following administration of a FVIII/VWF concentrate (Haemate® P). Skin prick tests and intradermal tests were performed for diagnostic evaluation and were found to be negative. The hypersensitivity reaction was assessed as an immediate type and non-IgE-mediated reaction. As there were no alternative treatment options, we decided to perform desensitization. A 14-step desensitization protocol was successfully administered. The patient is now able to self-administer Haemate® P at home three times a week. This case highlights the importance of desensitization and multidisciplinary approach in the case of drug hypersensitivity in patients with VWD Type 3. Our desensitization protocol with FVIII/VWF concentrate is highly effective and safe.
Left Renal Artery Thrombosis in a Patient With von Willebrand Disease Type 3 and Factor V Leiden Heterozygosity.
There are a few cases of combined thrombotic and hemophilic disorders coexisting in patients that have been published in the literature, such as factor V Leiden (FVL) with von Willebrand disease (VWD) type 1, and a report of a patient with VWD type 3, protein C and antithrombin III deficiency, and venous thromboses, but no reports in the literature were found of a patient with VWD type 3 and FVL and arterial thromboses. VWD type 3 is the most severe form because it results in nonexistent levels of von Willebrand factor (VWF) and is believed to be protective against arterial thromboses. We present a unique case of a 41-year-old man with a known history of VWD type 3 who presented with left renal artery thrombosis and a large left renal infarct, who was subsequently diagnosed with FVL heterozygosity. Both FVL and VWD type 3 can coexist in a patient. These patients require special considerations in treatment that have not been well documented throughout the literature.
Genetically Confirmed Dual Hematologic Disorder: A Case of β-Thalassemia with Frameshift Mutation and Type 3 von Willebrand Disease in a Pediatric Patient.
The coexistence of β-thalassemia major and Type 3 von Willebrand Disease (VWD) is an exceptionally rare clinical phenomenon. We describe a 3-year-old female with genetically confirmed β-thalassemia major due to an HBB frameshift mutation (exons 8-9) and Type 3 VWD with von Willebrand factor (VWF) antigen at 1.8%. Clinically, she presented with recurrent epistaxis, anemia, and transfusion dependence. Serial laboratory investigations revealed persistent microcytic hypochromic anemia, iron overload (ferritin 1778 ng/mL), and markedly low VWF antigen. Management included red blood cell transfusions, chelation, hydroxyurea, vitamin supplementation, and supportive care, while balancing the bleeding risks from VWD. This report underscores the diagnostic challenge and therapeutic complexity of overlapping congenital anemia and bleeding disorder. Multidisciplinary care and genetic testing were pivotal in confirming the diagnosis and guiding management.
Publicações recentes
Left Renal Artery Thrombosis in a Patient With von Willebrand Disease Type 3 and Factor V Leiden Heterozygosity.
A phase 1, open-label study to assess the pharmacokinetics, safety, and tolerability of a single intravenous injection of efanesoctocog alfa in adults with type 2N or type 3 von Willebrand disease.
A Rare Case of Klippel Trenaunay Syndrome with Von Willebrand Factor Deficiency and Multiple Accessory Spleens: A Case Report and Brief Literature Review.
Colon cancer surgery in von Willebrand disease type 3 setting triggering vascular abnormalities on bowel anastomosis.
Pharmacokinetic-Pharmacodynamic Comparison of Recombinant and Plasma-Derived von Willebrand Factor in Patients with von Willebrand Disease Type 3.
📚 EuropePMC28 artigos no totalmostrando 93
Successful desensitization with FVIII/Von Willebrand Factor concentrate in Type III Von Willebrand Disease.
Allergologia et immunopathologiaLeft Renal Artery Thrombosis in a Patient With von Willebrand Disease Type 3 and Factor V Leiden Heterozygosity.
CureusGenetically Confirmed Dual Hematologic Disorder: A Case of β-Thalassemia with Frameshift Mutation and Type 3 von Willebrand Disease in a Pediatric Patient.
HemoglobinA phase 1, open-label study to assess the pharmacokinetics, safety, and tolerability of a single intravenous injection of efanesoctocog alfa in adults with type 2N or type 3 von Willebrand disease.
Journal of thrombosis and haemostasis : JTHManagement of gastrointestinal bleeding in a patient with type 3 von Willebrand disease and inhibitors to von Willebrand factor: a case report.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisA VWF missense variant in Havanese dogs with type 3 von Willebrand disease.
Animal geneticsA 4-Week-Old Female Infant with Type 3 von Willebrand Disease Presenting with Nosebleeds and Uncontrolled Bleeding Following Surgical Frenectomy: A Clinical Case.
The American journal of case reportsDiagnosis of type III von Willebrand disease in a standard dachshund.
The Journal of small animal practicePrevalence and characterization of anti-VWF antibodies in a population of patients with type 3 VWD.
Blood advancesUnravelling the spectrum of von Willebrand factor variants in quantitative von Willebrand disease: results from a German cohort study.
Journal of thrombosis and haemostasis : JTHThe impact of von Willebrand factor on fibrin formation and structure unveiled with type 3 von Willebrand disease plasma.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisEmicizumab in Type 3 von Willebrand Disease: Report of a Case with an Alloantibody and Literature Review.
Seminars in thrombosis and hemostasisEfficacy of emicizumab therapy in two adult patients with type 3 von Willebrand disease.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisA Rare Case of Klippel Trenaunay Syndrome with Von Willebrand Factor Deficiency and Multiple Accessory Spleens: A Case Report and Brief Literature Review.
Advanced biomedical researchDiagnosis and treatment of von Willebrand disease in 2024 and beyond.
Haemophilia : the official journal of the World Federation of HemophiliaGenetic variations of type 2 and type 3 von Willebrand diseases in Thailand.
Journal of clinical pathologyAbdominoplasty in a Patient With Type 3 von Willebrand Disease: A Case Report.
Annals of plastic surgeryColon cancer surgery in von Willebrand disease type 3 setting triggering vascular abnormalities on bowel anastomosis.
Research and practice in thrombosis and haemostasisPharmacokinetic-Pharmacodynamic Comparison of Recombinant and Plasma-Derived von Willebrand Factor in Patients with von Willebrand Disease Type 3.
Journal of blood medicineReported prevalence of von Willebrand disease worldwide in relation to income classification.
Haemophilia : the official journal of the World Federation of HemophiliaVon Willebrand Disease: Gaining a global perspective.
Haemophilia : the official journal of the World Federation of HemophiliaEfficacy of platelet-inspired hemostatic nanoparticles on bleeding in von Willebrand disease murine models.
BloodSwitch to pdVWF:pdFVIII concentrate for prophylaxis in a paediatric patient with Type 3 von Willebrand disease: a case report.
Hematology (Amsterdam, Netherlands)An evaluation of von Willebrand factor (recombinant) therapy for adult patients living with severe type 3 von Willebrand disease.
Expert review of hematologyProphylaxis with recombinant von Willebrand factor in patients with type 3 von Willebrand disease: Results of a post hoc analysis from a phase 3 trial.
European journal of haematologyvon Willebrand factor neutralizing and non-neutralizing alloantibodies in 213 subjects with type 3 von Willebrand disease enrolled in 3WINTERS-IPS.
Journal of thrombosis and haemostasis : JTHProtein S gene mutation c.946C > T (p.R316C) contributed to ischemic stroke in a man with von Willebrand disease type 3 caused by two novel VWF gene mutations, c.2328delT (p.A778Lfs* 23) and c.6521G > T (p.C2174F).
Clinical case reportsNoninvasive fetal genotyping of single nucleotide variants and linkage analysis for prenatal diagnosis of monogenic disorders.
Human genomicsSixth Åland Island Conference on von Willebrand disease.
Haemophilia : the official journal of the World Federation of HemophiliaLongitudinal bleeding assessment in von Willebrand disease utilizing an interim bleeding score.
Journal of thrombosis and haemostasis : JTHGenetic Alterations, DNA Methylation, Alloantibodies and Phenotypic Heterogeneity in Type III von Willebrand Disease.
GenesCombination therapy with von Willebrand factor concentrate plus recombinant factor VIII during cesarean section in a patient with type 3 von Willebrand disease and a low inhibitor titer: a case report.
International journal of hematologyVWF-Gly2752Ser, a novel non-cysteine substitution variant in the CK domain, exhibits severe secretory impairment by hampering C-terminal dimer formation.
Journal of thrombosis and haemostasis : JTHEmicizumab enhances thrombus formation in vitro under high shear flow conditions in whole blood from patients with type 1 and type 3 von Willebrand disease.
Haemophilia : the official journal of the World Federation of Hemophiliavon Willebrand factor propeptide variants lead to impaired storage and ER retention in patient-derived endothelial colony-forming cells.
Journal of thrombosis and haemostasis : JTHMolecular pathogenesis and heterogeneity in type 3 VWD families in U.S. Zimmerman program.
Journal of thrombosis and haemostasis : JTHParathyroid Adenoma in a Young Girl with Type 3 von Willebrand Disease: Comment on "Cancers in Patients with von Willebrand Disease".
Seminars in thrombosis and hemostasisVon Willebrand factor propeptide and pathophysiological mechanisms in European and Iranian patients with type 3 von Willebrand disease enrolled in the 3WINTERS-IPS study.
Journal of thrombosis and haemostasis : JTHLaboratory characterization of obligate carriers of type 3 von Willebrand disease with a potential role for Platelet Function Analyzer (PFA-200).
International journal of laboratory hematologySocial participation is reduced in type 3 Von Willebrand disease patients and in patients with a severe bleeding phenotype.
Haemophilia : the official journal of the World Federation of HemophiliaGenotypes of European and Iranian patients with type 3 von Willebrand disease enrolled in 3WINTERS-IPS.
Blood advancesAdolescent With von Willebrand Disease Type 3 Spontaneous Abdominal Hemorrhage.
Journal of emergency nursingPhage display broadly identifies inhibitor-reactive regions in von Willebrand factor.
Journal of thrombosis and haemostasis : JTHMonitoring of von Willebrand factor inhibitors in patients with type 3 von Willebrand disease using a quantitative assay.
Haemophilia : the official journal of the World Federation of HemophiliaVascular abnormalities in patients with von Willebrand disease: A scoping review.
Journal of thrombosis and haemostasis : JTHVariants p.Pro2063Ser and p.Arg324* co-segregate in type 3 von Willebrand disease patients from Southern Brazil.
Haemophilia : the official journal of the World Federation of HemophiliaInhibitor development in patients with type 3 Von Willebrand disease, a comprehensive study on 99 Iranian patients.
Haemophilia : the official journal of the World Federation of HemophiliaRisk of perinatal intracranial hemorrhage and role of prenatal genetic testing in individuals with type 3 von Willebrand disease.
Journal of thrombosis and haemostasis : JTHEndothelial Function in Patients With Von Willebrand Disease.
Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisPrimary thromboprophylaxis in a patient with type 3 von Willebrand disease and severe COVID-19 infection.
Haemophilia : the official journal of the World Federation of HemophiliaThe homozygous variant p.Gln1311* in exon 28 of VWF is associated with the development of alloantibodies in 3 unrelated patients with type 3 VWD.
Haemophilia : the official journal of the World Federation of HemophiliaA Combination of Two Variants p. (Val510 =) and p. (Pro2145Thrfs * 5), Responsible for von Willebrand Disease Type 3 in a Caribbean Patient.
TH open : companion journal to thrombosis and haemostasisKinetics of Platelet Adhesion to Protein-Coated Surface in Whole Blood Samples at High Flow Rates.
Bulletin of experimental biology and medicineBleeding symptoms in patients diagnosed as type 3 von Willebrand disease: Results from 3WINTERS-IPS, an international and collaborative cross-sectional study.
Journal of thrombosis and haemostasis : JTHPresentation and diagnosis of patients with type 3 von Willebrand disease in resources-limited laboratory.
Hematology/oncology and stem cell therapyTwo cases of von Willebrand disease type 3 in consanguineous Chinese families.
Molecular genetics & genomic medicineType 3 von Willebrand Disease in Pregnancy: A Systematic Literature Review.
American journal of perinatologyHigher rates of bleeding and use of treatment products among young boys compared to girls with von Willebrand disease.
American journal of hematologyCharacterization of the mutation spectrum in a Pakistani cohort of type 3 von Willebrand disease.
Haemophilia : the official journal of the World Federation of HemophiliaHow I manage severe von Willebrand disease.
British journal of haematologySevere recurrent endometriomas in a young woman with congenital von Willebrand disease.
Gynecological endocrinology : the official journal of the International Society of Gynecological EndocrinologyA Novel Case of Compound Heterozygous Type 3 Von Willebrand Disease.
Annals of clinical and laboratory scienceGastrointestinal angiodysplasia in two patients with type 3 von Willebrand disease.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisIntravenous immunoglobulin treatment in a type 3 von Willebrand disease patient with alloantibodies and a life-threatening gastrointestinal bleed.
Haemophilia : the official journal of the World Federation of HemophiliaPlatelets compensate for poor thrombin generation in type 3 von Willebrand disease.
PlateletsJoint Mobility and Physical Function of Danish Hemophilia Patients: A Three-Wave Panel Study Spanning 24 Years.
Acta haematologicaType-3 von Willebrand disease in India-Clinical spectrum and molecular profile.
Haemophilia : the official journal of the World Federation of HemophiliaLiver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome.
Revista espanola de enfermedades digestivasType 3 von Willebrand disease mistaken for moderate haemophilia A: a lesson still to be learned.
Haemophilia : the official journal of the World Federation of HemophiliaIs gingival bleeding a symptom of type 2 and 3 von Willebrand disease?
PloS oneA 12.3-kb Duplication Within the VWF Gene in Pigs Affected by Von Willebrand Disease Type 3.
G3 (Bethesda, Md.)[Genetic and prenatal diagnosis of a pedigree affected with type 3 von Willebrand disease].
Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical geneticsWhole blood ristocetin-activated platelet impedance aggregometry (Multiplate) for the rapid detection of Von Willebrand disease.
Thrombosis and haemostasis[Von Willebrand disease type 3 falsely diagnosed as hemophilia A: a case report].
Revue medicale de BruxellesDiscrepant platelet and plasma von Willebrand factor in von Willebrand disease patients with p.Pro2808Leufs*24.
Journal of thrombosis and haemostasis : JTHAutosomal recessive inherited bleeding disorders in Pakistan: a cross-sectional study from selected regions.
Orphanet journal of rare diseasesDiagnosing von Willebrand disease: genetic analysis.
Hematology. American Society of Hematology. Education ProgramMonitoring of coagulation factor therapy in patients with von Willebrand disease type 3 using a microchip flow chamber system.
Thrombosis and haemostasisSkin testing, graded challenge, and desensitization to von Willebrand factor (VWF) products in type III von Willebrand disease (VWD).
The journal of allergy and clinical immunology. In practiceCharacterization of aberrant splicing of von Willebrand factor in von Willebrand disease: an underrecognized mechanism.
BloodProphylaxis in von Willebrand Disease: Coming of Age?
Seminars in thrombosis and hemostasisVon Willebrand factor regulates complement on endothelial cells.
Kidney internationalCanine models of inherited bleeding disorders in the development of coagulation assays, novel protein replacement and gene therapies.
Journal of thrombosis and haemostasis : JTHSelf-infusion of prophylaxis: evaluating the quality of its performance and time needed.
Haemophilia : the official journal of the World Federation of HemophiliaBaseline factor VIII plasma levels and age at first bleeding in patients with severe forms of von Willebrand disease.
Haemophilia : the official journal of the World Federation of HemophiliaLiver Transplantation in Type III von Willebrand Disease.
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant SurgeonsCongenital Type III von Willebrand's disease unmasked by hypothyroidism in a Shetland sheepdog.
The Canadian veterinary journal = La revue veterinaire canadienneAngiogenic characteristics of blood outgrowth endothelial cells from patients with von Willebrand disease.
Journal of thrombosis and haemostasis : JTHSuccessful aortic aneurysm repair in a woman with severe von Willebrand (type 3) disease.
Case reports in hematologyManagement of pregnancy in type 3 von Willebrand disease with additional platelet dysfunction.
Haemophilia : the official journal of the World Federation of HemophiliaSpectrum of Von Willebrand's disease in Punjab: clinical features and types.
Journal of Ayub Medical College, Abbottabad : JAMCNo evidence for a direct effect of von Willebrand factor's ABH blood group antigens on von Willebrand factor clearance.
Journal of thrombosis and haemostasis : JTHAssessment of the olfactory function in Italian patients with type 3 von Willebrand disease caused by a homozygous 253 Kb deletion involving VWF and TMEM16B/ANO2.
PloS oneAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Doença de Von Willebrand, tipo 3.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Doença de Von Willebrand, tipo 3
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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.
- A phase 1, open-label study to assess the pharmacokinetics, safety, and tolerability of a single intravenous injection of efanesoctocog alfa in adults with type 2N or type 3 von Willebrand disease.
- Management of gastrointestinal bleeding in a patient with type 3 von Willebrand disease and inhibitors to von Willebrand factor: a case report.Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis· 2025· PMID 40549426mais citado
- Successful desensitization with FVIII/Von Willebrand Factor concentrate in Type III Von Willebrand Disease.
- Left Renal Artery Thrombosis in a Patient With von Willebrand Disease Type 3 and Factor V Leiden Heterozygosity.
- Genetically Confirmed Dual Hematologic Disorder: A Case of β-Thalassemia with Frameshift Mutation and Type 3 von Willebrand Disease in a Pediatric Patient.
- A Rare Case of Klippel Trenaunay Syndrome with Von Willebrand Factor Deficiency and Multiple Accessory Spleens: A Case Report and Brief Literature Review.
- Colon cancer surgery in von Willebrand disease type 3 setting triggering vascular abnormalities on bowel anastomosis.
- Pharmacokinetic-Pharmacodynamic Comparison of Recombinant and Plasma-Derived von Willebrand Factor in Patients with von Willebrand Disease Type 3.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:166096(Orphanet)
- OMIM OMIM:277480(OMIM)
- MONDO:0010191(MONDO)
- GARD:17025(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q32146577(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
