Grupo heterogêneo de neoplasias linfóides malignas de origem de células B caracterizadas histologicamente pela presença de células Hodgkin e Reed-Sternberg (HRS) na grande maioria dos casos. Existem dois subtipos distintos: linfoma de Hodgkin com predominância de linfócitos nodulares e linfoma de Hodgkin clássico. O linfoma de Hodgkin envolve principalmente os gânglios linfáticos.
Introdução
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Grupo heterogêneo de neoplasias linfóides malignas de origem de células B caracterizadas histologicamente pela presença de células Hodgkin e Reed-Sternberg (HRS) na grande maioria dos casos. Existem dois subtipos distintos: linfoma de Hodgkin com predominância de linfócitos nodulares e linfoma de Hodgkin clássico. O linfoma de Hodgkin envolve principalmente os gânglios linfáticos.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 14 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 39 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: Multigenic/multifactorial.
Involved in pinching off the separated nuclei at the cleavage furrow and in cytokinesis (PubMed:20107318). Required for mitotic integrity and maintenance of chromosomal stability. Protects cells against mitotic errors, centrosomal amplification, micronucleus formation and aneuploidy. Plays a key role of midbody function involving abscission of the daughter cells during cytokinesis and appropriate chromosomal and nuclear segregation into the daughter cells (PubMed:22988245, PubMed:23713010)
CytoplasmMidbody
Lymphoma, Hodgkin, classic
A malignant disease characterized by progressive enlargement of the lymph nodes, spleen and general lymphoid tissue, and the presence of large, usually multinucleate, cells (Reed-Sternberg cells). Reed-Sternberg cells compose only 1-2% of the total tumor cell mass. The remainder is composed of a variety of reactive, mixed inflammatory cells consisting of lymphocytes, plasma cells, neutrophils, eosinophils and histiocytes.
Medicamentos aprovados (FDA)
3 medicamentos encontrados nos registros da FDA americana.
Variantes genéticas (ClinVar)
18 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 9 variantes classificadas pelo ClinVar.
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 — Linfoma de Hodgkin
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 14.840
Proteome-wide Mendelian randomization identifies protein associations and therapeutic targets for B-cell malignancy.
Despite therapeutic advances in B-cell malignancies, many patients continue to experience relapse or refractory disease, highlighting an unmet need for novel drug targets. The high attrition rate of drug development programs, however, represents a productivity-limiting step. To prioritize candidate drug targets, we used Mendelian randomization to evaluate causal associations between 2923 circulating proteins and 6 B-cell malignancies (22 922 cases and 388 978 controls). We identified 27 protein-disease associations, including TNFSF13 (APRIL) and TNFRS13B (TACI) in multiple myeloma, CD40 in Hodgkin lymphoma, and FAS in chronic lymphocytic leukemia. All results are interactively accessible via our R/Shiny application (https://software.icr.ac.uk/app/mrcan). Integrating single-cell RNA sequencing from 183 355 hematolymphoid cells, Bayesian colocalization, and clinical trial evidence, we prioritized 23 proteins as candidate drug targets. This study demonstrates the potential of human genetics to guide therapeutic discovery for B-cell neoplasia.
METTL3 stabilizes FASN mRNA by mediating m6A modification to promote malignant progression of diffuse large B-cell lymphoma.
Diffuse large B-cell lymphoma (DLBCL) is an aggressive subtype of non-Hodgkin lymphoma. Fatty acid synthase (FASN) is a key lipogenic enzyme implicated in tumor progression, but its regulation in DLBCL is poorly understood. The function of FASN in DLBCL was explored using database analysis, clinical sample analysis, and cellular phenotyping experiments. Candidate m6A sites on FASN mRNA were predicted using the SRAMP database. Bioinformatics analyses and experimental techniques were conducted to elucidate the role of methyltransferase-like 3 (METTL3) on cell phenotypes and its regulation of FASN. The findings were further confirmed in mice models and clinical sample analyses. FASN was highly expressed in DLBCL and positively correlated with poor prognosis. FASN knockdown inhibited the malignant phenotypes in DLBCL cells by suppressing the PI3K/AKT and MAPK/ERK signaling pathways, as well as promoting endoplasmic reticulum (ER) stress. The analysis of FASN mRNA revealed the presence of m6A modification sites, and a positive correlation was identified between METTL3 and FASN. The impact of METTL3 knockdown on the malignant phenotypes of DLBCL cells was consistent with the effects induced by FASN knockdown, whereas METTL3 overexpression reversed the effects of FASN knockdown. Mechanistically, METTL3 stabilized FASN expression by mediating m6A modification of FASN mRNA, thereby facilitating DLBCL progression. METTL3 stabilizes FASN expression through m6A modification, thereby facilitating the DLBCL progression by activating the PI3K/AKT and MAPK/ERK signaling pathways and inhibiting the ER stress response pathway. The METTL3/FASN axis represents a potential therapeutic target, especially in METTL3/FASN-high DLBCL subgroups.
Clinical Manifestations of Primary CNS T-Cell Lymphoma: A Retrospective Study of Histopathologic, Molecular, and Neuroimaging Features.
Primary CNS T-cell lymphoma (PCNSTL) is an extremely rare and aggressive form of non-Hodgkin lymphoma. Diagnosis is often challenging because of the nonspecific clinical presentation, which can lead to delays in treatment. This study aims to analyze the clinical, histopathologic, and neuroimaging characteristics of PCNSTL. In this retrospective, multicentric cohort study, histologically confirmed PCNSTL cases without evidence of systemic disease were selected from pathology databases at 3 academic neuro-oncology centers (University Hospital of Munich, University Hospital of Heidelberg, Massachusetts General Hospital in Boston) during the period from 2008 to 2024. Retrospective data, including demographics, histopathology, immunophenotyping, multimodal MRI, and PET imaging, treatment lines, and outcome data were extracted from medical records and analyzed. We evaluated 16 patients (11 male) with a median age of 50 years (19-76 years) and a median Karnofsky performance status of 80% (20%-100%). T-cell clonality was confirmed in 9/15 (60%) tested patients with typical T-cell receptor gene rearrangement patterns. MRI scans at primary diagnosis showed predominantly supratentorial parenchymal lesions with prominent contrast enhancement in 14/16 (87.5%) and in more than one-third multifocal lesions (7/16, 43.75%). High intratumoral susceptibility signal was frequently observed (7/16, 43.75%). [18F]fluorodeoxyglucose-PET and [18F]fluoroethyltyrosine-PET imaging revealed only low to moderate tracer uptake in 7/8 examined patients (87.5%). Median progression-free survival was 4 months, and median overall survival was 97.5 months. Although treatment protocols varied, the use of methotrexate (MTX)-based chemotherapy combined with autologous stem-cell transplant (ASCT) was associated with most favorable outcome (95% CI 2-87, p < 0.0275). In 1 case of ALK1-positive PCNSTL, persistent complete remission was achieved after treatment with the ALK-inhibitor lorlatinib. Although PCNSTL is exceptionally rare, we identified distinct neuroimaging patterns showing highly aggressive features on MRI but hypometabolic PET imaging, which may assist in identifying future PCNSTL cases. Despite the limited cohort size, our findings suggest that MTX-based chemotherapy with ASCT may translate into favorable outcome. We report on an ALK1-positive PCNSTL case with sustained complete remission after targeted therapy with lorlatinib.
Association of MMP-2 and MMP-9 Polymorphisms and the Pathogenesis of Childhood Burkitt's Lymphoma.
Burkitt lymphoma (BL) is a highly aggressive and invasive non-Hodgkin lymphoma derived from germinal center B cells and represents one of the most common childhood malignancies. Matrix metalloproteinases (MMPs) play a critical role in cancer progression by remodeling the extracellular matrix and modulating the tumor microenvironment. In advanced stages, MMPs facilitate tumor invasion and metastasis through the degradation of extracellular matrix components. This study aimed to investigate the association between MMP-2 and MMP-9 gene polymorphisms and the pathogenesis of childhood BL. This study was conducted at the Pediatric Oncohematology Center of the University of Pernambuco between 2021 and 2023 and included patients of both sexes aged 1 to 18 years diagnosed with BL between 1993 and 2023. Genomic DNA was extracted from peripheral blood samples using the salting-out method. Polymorphisms were identified by real-time polymerase chain reaction (PCR) using the TaqMan system. A total of 56 patients diagnosed with BL were analyzed; most were male, with a mean age of 7.1 years. The overall survival rate was 92.6%. A protective effect of the mutant TT genotype was observed compared to the wild-type CC genotype (OR = 0.0562; 95% CI: 0.0068-0.4644; p = 0.0016) for the MMP-2 polymorphism. Furthermore, individuals carrying the mutant G allele showed a positive association with BL compared to the wild-type A allele (G vs. A: OR = 1.9920; 95% CI: 1.1812-3.3594; p = 0.0130). These findings contribute to a better understanding of the genetic factors influencing BL susceptibility and support the need for further functional and large-scale studies to elucidate the mechanistic role of MMP-2 and MMP-9 polymorphisms in BL pathogenesis.
Real-World Outcomes of Allogeneic Stem Cell Transplantation in Relapsed Hodgkin Lymphoma: A Single-Center Experience from India.
Relapsed Hodgkin lymphoma (HL) after autologous stem cell transplantation (auto-HSCT) remains a major therapeutic challenge with few curative options. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) may provide durable disease control, yet outcome data from tertiary-care centres in India are limited. To evaluate real-world outcomes of allo-HSCT in patients with chemotherapy-sensitive relapsed HL at a tertiary care centre in North India. This retrospective study included patients with chemotherapy-sensitive relapsed HL who underwent allo-HSCT at the Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, between January 2017 and December 2023. Clinical data including baseline characteristics, transplant parameters, complications, and survival outcomes were reviewed. Descriptive statistics were used, and overall survival (OS) was estimated using the Kaplan-Meier method. Five patients (median age 18 years; range 14-30) with chemotherapy-sensitive relapse following auto-HSCT underwent allogeneic transplantation in complete remission (CR) after salvage therapy. Donors included haploidentical (n=3), matched unrelated (n=1), and matched sibling (n=1). Four patients received reduced-intensity conditioning; one received myeloablative conditioning. Graft-versus-host disease (GVHD) prophylaxis included post-transplant cyclophosphamide (PT-Cy) in four patients. Neutrophil and platelet engraftment occurred at median 15 and 16 days, respectively. All patients developed bacterial infections; one succumbed to Gram-negative sepsis. Acute grade III GVHD occurred in one patient, and two developed limited chronic GVHD. At a median follow-up of 44 months, four patients remained in continuous remission, with a 3-year OS of 80%. In Indian patients with chemotherapy-sensitive relapsed HL, allo-HSCT using reduced-intensity and reduced toxicity conditioning regimens and post-transplant cyclophosphamide-based regimens has proven feasible and effective, yielding encouraging survival outcomes within routine clinical practice.
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Medicina (Kaunas, Lithuania)Decoding NOTCH1: From T-Cell Development Guardian to Driver of Pediatric T-Cell Lymphoblastic Lymphoma.
International journal of molecular sciencesLipid Metabolism Reprogramming in Diffuse Large B-Cell Lymphoma (DLBCL): Mechanisms and Treatment Strategies.
CancersSecondary Neoplasm in Survivors of Childhood Hematological Malignancies-Systematic Review.
Children (Basel, Switzerland)Vegetarian diets and cancer risk: pooled analysis of 1.8 million women and men in nine prospective studies on three continents.
British journal of cancerAssociation Between Patient-Reported Outcomes Prior to Chimeric Antigen Receptor (CAR) T-Cell Therapy and Clinical Outcomes.
Transplantation and cellular therapyResolution of vanishing bile duct syndrome in a patient associated with refractory hodgkin lymphoma following Anti-PD-1 therapy: a case report and literature review.
Annals of hematologyExtranodal NK/T-Cell Lymphoma, Nasal Type, Presenting as an Isolated Oral Manifestation.
Dentistry journalImmune-related adverse events in the treatment of Hodgkin lymphoma with immune checkpoint inhibitors.
British journal of haematologyAssociations of serum lipid traits with DLBCL: a prospective cohort study from the UK Biobank.
Frontiers in nutritionCharacterization of JNJ-80948543 a novel CD79bxCD20xCD3 trispecific antibody for B-cell non-Hodgkin lymphoma.
HaematologicaAnti-programmed cell death protein 1-based salvage therapy for relapsed/refractory Hodgkin lymphoma: a multicenter real-world analysis.
HaematologicaIs it time to reduce the doxorubicin dosage in Hodgkin lymphoma therapy?
Haematologica[Primary mediastinal B-cell lymphoma presenting as cardiac tamponade: the importance of a multimodality imaging approach].
Giornale italiano di cardiologia (2006)Long-term Health Outcomes in Older Blood or Marrow Transplantation Recipients: A Report from the BMTSS.
Blood advancesThe surgery for the patients with intestinal non‑Hodgkin lymphomas: a nationwide study.
Annals of medicineUnmasking Diffuse Large B-Cell Lymphoma: A Challenging Case of Acute Cardiac Tamponade in the Setting of Right-Sided Heart Mass Invasion.
Journal of investigative medicine high impact case reportsA pharmacovigilance study of rituximab-associated adverse events in immune-mediated kidney diseases and transplant-related kidney diseases.
International journal of surgery (London, England)Demographic and clinical profiles of patients with primary and second primary cancers: identifying biomarkers of sensitivity to radiotherapy-induced cancer.
International journal of radiation biologyGeographical variation in Hodgkin lymphoma incidence patterns by histological subtype and sex: a population-based analysis of cancer registry data.
International journal of surgery (London, England)Characteristics and Outcomes of Patients with Hodgkin Lymphoma with Paraneoplastic Manifestations.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionExpression of Programmed Death Ligand 1 (PD-L1) in Classical Hodgkin Lymphoma- Study from a Tertiary Care Cancer Centre in South India.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionResearch on the correlation and potential mechanism of PKCδ expression with efficacy and prognosis in diffuse large B-cell lymphoma.
Frontiers in oncologySafety and clinical outcomes of a first-in-human trial of point-of-care manufactured trispecific CAR T cells targeting CD19, CD20, and CD22.
Research squarePre-treatment endocrine-nutritional signatures predict clinical benefit from PD-1/PD-L1 blockade in hematologic malignancies.
Frontiers in nutritionInfliximab Monotherapy Versus Infliximab and Azathioprine Combination Therapy in Patients with Ulcerative Colitis: A Cost-Effectiveness Analysis.
Digestive diseases and sciencesMachine learning based on clinical and gene expression data assists in survival prediction and treatment optimization for diffuse large B-Cell lymphoma patients.
Annals of hematologyPrognostic impact of treatment-related and geriatric factors in older patients with classic Hodgkin lymphoma: A real-life cohort study.
British journal of haematologyPatient- and caregiver-reported barriers to radiotherapy for cancer in sub-Saharan Africa-A survey of population-based registries.
International journal of cancerGenetic variations of STAT3 gene and their role in hodgkin lymphoma incidence.
Molecular biology reportsDescriptive Case Series of Childhood Lymphomas Treated at the Children's Hospital of Mexico.
Pediatric reportsPrimary Bone Lymphoma of the Jaw Masquerading as Infection and Delaying Treatment.
Hematology reportsCladosporium cladosporioides Fungemia in a Patient with Non-Hodgkin Lymphoma: An Extremely Rare Case and Review of the Literature.
Reports (MDPI)The burden of cancers and their variations across the states of US the global burden of disease study 1990-2021.
International journal of surgery (London, England)The evolving role of [¹⁸F]FDG PET/CT in reducing or replacing biopsy in selected malignancies: A narrative literature review.
Journal of medical imaging and radiation sciencesPrimary Central Nervous System Lymphoma With Testicular and Prostatic Involvement: A Case Report.
CureusEarly-Stage Hodgkin Lymphoma: Time for Novel Agents?
Hematology/oncology clinics of North AmericaClassic Hodgkin Lymphoma in the Older Adult: What's New?
Hematology/oncology clinics of North AmericaAn Unusual Case of Classic Hodgkin Lymphoma With Strong Expression of B-Cell Markers.
International journal of laboratory hematologyPotential of Exercise for Prevention of Cardiovascular Disease in Survivors of Childhood Hodgkin Lymphoma.
JACC. CardioOncologyObstructive Jaundice Revealing Primary Diffuse Large B-Cell Lymphoma of the Ampulla of Vater in a Child: A Case Report.
Case reports in oncologyEfficacy of immune checkpoint inhibitors in paediatric, adolescent and young adults with primary refractory or relapsed classical Hodgkin lymphoma: A national multicentre real-world study.
British journal of haematologyType 2 diabetes and risk of non-Hodgkin lymphoma and multiple myeloma: a pooled analysis.
JNCI cancer spectrum2025 Report on the Mantle Cell Lymphoma Scientific Consortium and Workshop Hosted by the Lymphoma Research Foundation.
Oncology (Williston Park, N.Y.)Second Primary Subglottic Laryngeal Carcinoma After Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone (CHOP) Chemotherapy for Non-Hodgkin Lymphoma.
CureusRole of ¹⁸F-FDG PET Metabolic Parameters in Treatment Response Evaluation and Prognostic Assessment of Hodgkin Lymphoma: A Retrospective Analysis.
Turkish journal of haematology : official journal of Turkish Society of HaematologyOptimizing the Role of Checkpoint Inhibitors in the Management of Hodgkin Lymphoma.
Journal of the National Comprehensive Cancer Network : JNCCNPuzolcabtagene Autoleucel: Pediatric First Approval.
Paediatric drugsChallenges in delineating B-cell lymphoma: A case of plasmablastic lymphoma mimicking Burkitt lymphoma.
Journal of oral and maxillofacial pathology : JOMFPClinical characteristics, treatment patterns, and survival in mantle cell lymphoma: a real-world cohort.
Frontiers in oncologyHigh-Resolution Spatial Transcriptomic Characterization of Syncytial Variant of Nodular Sclerosis Classical Hodgkin Lymphoma.
Laboratory investigation; a journal of technical methods and pathologyA Call for Compassion: How You Can Help Get Multiple Myeloma Added to the Social Security Administration's Compassionate Allowances List.
Clinical lymphoma, myeloma & leukemiaIndividualized physical activity program for older adults undergoing chemotherapy for hematologic malignancies.
Journal of geriatric oncologyUnlocking the Secrets of Regulated Cell Death in Large B-Cell Lymphoma Beyond Apoptosis: Signaling Pathways and Therapeutic Options.
International journal of molecular sciencesImmune Checkpoint Blockade in Hematological Malignancies: Current Status and Future Directions.
CancersHodgkin Lymphoma-The Effect of Chemotherapy on Gonadal Function and Fertility Is Strongly Related to the Treatment Regimen, Age, and Sex: A Systematic Review and Meta-Analysis.
CancersLong-term follow-up demonstrates the curative potential of dual CD19/CD22 CAR-T-cell therapy alone or combined with autologous stem cell transplantation in TP53-altered relapsed/refractory B-cell non-Hodgkin lymphoma.
Signal transduction and targeted therapyPrognostic Impact of CD57⁺ Natural Killer Cells and CD138⁺ Plasma Cells as Minor Components of the Tumor Microenvironment in Mixed Cellularity Classical Hodgkin Lymphoma.
Applied immunohistochemistry & molecular morphology : AIMMThe Modern Role of Radiation Therapy in Classic Hodgkin Lymphoma.
Hematology/oncology clinics of North AmericaFollicular lymphoma transformed to classic Hodgkin lymphoma.
BloodGlobal trends and inequities in childhood cancer burden from 1990 to 2021, with projections to 2040: a Global Burden of Disease study.
International journal of surgery (London, England)The predictive value of interleukin-2 receptor and prognostic nutritional index in patients with diffuse large B-cell lymphoma.
Frontiers in medicineIncidence of de novo Lymphoproliferative Disorders and Hematological Malignancies in Liver Transplant Recipients: An Updated Meta-Analysis.
Saudi journal of medicine & medical sciencesParaneoplastic reactive perforating collagenosis in setting of Hodgkin lymphoma and prior marginal zone lymphoma.
JAAD case reportsDisease burden of hematological malignancies worldwide, in China and in the United States based on the GLOBOCAN 2022 and Global Burden of Disease 2021 data.
Chinese medical journalHodgkin Lymphoma, Version 1.2026, NCCN Clinical Practice Guidelines In Oncology.
Journal of the National Comprehensive Cancer Network : JNCCNEvaluating Vulnerable Elders Survey-13 and FIL Geriatric Assessment in Older Adults With Aggressive Lymphomas.
Journal of the National Comprehensive Cancer Network : JNCCNLong-term clinical outcomes of patients with localized primary ocular adnexal mucosa-associated lymphoid tissue lymphoma.
International journal of hematologyAssociações
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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.
- Proteome-wide Mendelian randomization identifies protein associations and therapeutic targets for B-cell malignancy.
- METTL3 stabilizes FASN mRNA by mediating m6A modification to promote malignant progression of diffuse large B-cell lymphoma.
- Clinical Manifestations of Primary CNS T-Cell Lymphoma: A Retrospective Study of Histopathologic, Molecular, and Neuroimaging Features.
- Association of MMP-2 and MMP-9 Polymorphisms and the Pathogenesis of Childhood Burkitt's Lymphoma.
- Real-World Outcomes of Allogeneic Stem Cell Transplantation in Relapsed Hodgkin Lymphoma: A Single-Center Experience from India.
- Diffuse Gastrointestinal Polyposis Revealing Mantle Cell Lymphoma: A Case Highlighting a Diagnostic Pitfall.
- Extranodal nasal-orbital communicating lesions NK/T cell lymphoma with ocular symptoms as the initial manifestation misdiagnosed as sinusitis and orbital cellulitis: a case report and literature review.
- Occult obstructive sleep apnea in survivors of Hodgkin lymphoma following radiation therapy: an atypical and under-recognized phenotype.
- Efficacy of bepotastine compared with hydroxyzine in preventing rituximab-induced infusion-related reactions in non-hodgkin lymphoma patients: a phase II, double-blind, multicenter, and randomized trial.
- Lenalidomide Plus Rituximab for Relapsed/Refractory Indolent Non-Hodgkin Lymphoma: 5-Year Follow-Up and Subgroup Analyses From the Phase III AUGMENT Trial.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98293(Orphanet)
- MONDO:0004952(MONDO)
- GARD:2714(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q209369(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
