Linfoma de células B maduras, geralmente indolente, que surge da zona marginal dos tecidos linfóides. É caracterizada pela presença de linfócitos atípicos de pequeno a médio porte. É composto por três entidades, de acordo com os locais anatômicos envolvidos: linfoma extranodal de células B da zona marginal do tecido linfóide associado à mucosa, que afeta locais extranodais (mais frequentemente estômago, pulmão, pele e anexos oculares); linfoma de células B da zona marginal nodal, que afeta os gânglios linfáticos sem evidência de doença extranodal; e linfoma de células B da zona marginal esplênica, que afeta o baço e os gânglios linfáticos hilares esplênicos, a medula óssea e, frequentemente, o sangue periférico.
Introdução
O que você precisa saber de cara
O linfoma da zona marginal é um tipo de câncer de evolução geralmente lenta que afeta células do sistema de defesa (células B maduras) em adultos. Ele pode surgir em órgãos como o estômago, nos gânglios linfáticos ou no baço, provocando inchaço nos gânglios, cansaço, febre e dor no abdômen. No Brasil, a condição não conta com um protocolo clínico (PCDT) próprio na lista oficial de doenças raras do Ministério da Saúde, sendo acompanhada diretamente pela rede oncológica do SUS. Existem opções de tratamento aprovadas no exterior e dezenas de estudos clínicos em andamento no mundo avaliando novas terapias.
Linfoma de células B maduras, geralmente indolente, que surge da zona marginal dos tecidos linfóides. É caracterizada pela presença de linfócitos atípicos de pequeno a médio porte. É composto por três entidades, de acordo com os locais anatômicos envolvidos: linfoma extranodal de células B da zona marginal do tecido linfóide associado à mucosa, que afeta locais extranodais (mais frequentemente estômago, pulmão, pele e anexos oculares); linfoma de células B da zona marginal nodal, que afeta os gânglios linfáticos sem evidência de doença extranodal; e linfoma de células B da zona marginal esplênica, que afeta o baço e os gânglios linfáticos hilares esplênicos, a medula óssea e, frequentemente, o sangue periférico.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
+ 4 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 18 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
5 genes identificados com associação a esta condição.
Immunodeficiency 12
A primary immunodeficiency characterized by onset in infancy of recurrent bacterial and candidal infections resulting in bronchiectasis and growth delay. Manifestations include mastoiditis, aphthous ulcers, cheilitis, gingivitis, esophagitis, gastritis, duodenitis, and meningitis. Levels of absolute lymphocytes and serum immunoglobulins are normal, but specific antibody titers are low despite immunization, and T-cells show impaired proliferative responses to mitogens.
Medicamentos aprovados (FDA)
3 medicamentos encontrados nos registros da FDA americana.
Variantes genéticas (ClinVar)
472 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
21 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 — Linfoma da zona marginal
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Com ensaio aberto em 36 países. O ponto verde marca onde há vaga agora.
🟢 Recrutando agora
87 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
765 ensaios clínicos encontrados, 152 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.842
High-grade follicular B-cell lymphoma-associated C3 rapidly progressive glomerulonephritis: a rare association.
Rapidly progressive glomerulonephritis (GN) in the context of B-cell lymphoma is rare, with most cases involving diffuse-large B-cell lymphoma or marginal zone lymphoma. Kidney biopsies are infrequently performed in this population, further limiting diagnostic clarity. Here, we report a case of newly diagnosed follicular large B-cell lymphoma (FLBCL) presenting with a nephritic syndrome, characterised by haematuria, hypertension and heavy proteinuria, caused by C3 crescentic GN, requiring dialysis. The patient was treated with polatuzumab, bendamustine and rituximab, which was well tolerated. Renal function gradually improved, leading to dialysis discontinuation, coinciding with lymphoma remission. This case underscores the atypical presentation of FLBCL, including renal dysfunction as an early manifestation, and the importance of prompt diagnosis and targeted therapy.
Comprehensive analysis of mortality risk factors in low-grade B-cell lymphoma.
Low-grade B-cell lymphomas (LGBCLs) account for approximately 40% of non-Hodgkin lymphomas with low progression. LGBCL is divided into subgroups, which share common complications. Analyzing prognostic factors and mortality causes could improve patient survival; however, currently available models present limitations in discriminating the cause of death. Therefore, this study aimed to compare the prognostic factors and causes of death, such as secondary malignancies (SMs), aggressive histologic transformation (HT), and infectious complications, including coronavirus disease 2019 (COVID-19), in LGBCLs using a competing risk analysis. This retrospective analysis included 1,047 adults with LGBCLs (follicular lymphoma, 689; marginal zone lymphoma, 312; mantle cell lymphoma [MCL], 46) diagnosed between January 2011 and December 2022 across seven centers. Competing risk models were employed to estimate cumulative incidence rates of lymphoma progression-related and non-lymphoma-related mortality. Patients with SMs (3.8%) exhibited poorer overall survival than those without SMs, whereas HT and COVID-19 status did not impact survival outcomes in multivariate analysis. Analysis revealed an association of SMs, age > 60 years, male sex, pleural effusion, and elevated lactate dehydrogenase levels with worse non-lymphoma-related mortality. Moreover, age > 60 years, MCL, nodal MZL, and anemia were linked to poorer outcomes for lymphoma progression-related death. The management of non-lymphoma-related risk factors, such as through early SM detection, is crucial for improving the survival of patients with LGBCLs.
Field-testing the MALT-IPI in a single center real-world cohort of patients with extranodal marginal zone lymphoma.
The MALT-IPI is widely applied to estimate outcomes in patients with extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT). Although it was developed in the context of the IELSG-19 trial exclusively in patients treated with chlorambucil, rituximab or their combination, it is commonly used across virtually all disease settings. However, its applicability beyond contexts resembling the IELSG-19 trial remains debated, and several studies have proposed modifications to improve prognostic precision in EMZL. Here, we retrospectively analyzed 498 patients with newly diagnosed EMZL at the Medical University of Vienna with the dual objective of evaluating the prognostic performance of the MALT-IPI across clinical subgroups, and to assess whether incorporation of autoimmunity could refine risk stratification. Stratification by the MALT-IPI revealed significantly longer progression-free survival (PFS) and overall survival (OS) in low-risk patients compared with intermediate- and high-risk groups (p<0.001) in unselected patients. However, in subgroup analyses, its discriminatory capacity was restricted to patients with extragastric disease, particularly patients with EMZL of the ocular adnexa or the parotid gland or intestinal EMZL, as well as those receiving systemic therapy. Contrarily, patients with gastric EMZL or those managed with Helicobacter pylori eradication, local therapy or watch-and-wait approaches did not derive consistent prognostic separation. Incorporation of autoimmunity identified a small subgroup with inferior PFS and OS but provided limited incremental prognostic power beyond the established index in most patients. Our results support context-specific applicability of the MALT-IPI and highlight autoimmunity as a prognostically relevant factor in a small cohort of patients.
Lisocabtagene maraleucel in patients with relapsed or refractory marginal zone lymphoma (TRANSCEND FL): primary analysis results from the global, multicohort, single-arm, phase 2 study.
Effective treatments with deep and durable responses for relapsed or refractory marginal zone lymphoma (MZL) are lacking. The objective of the primary analysis from the MZL cohort of TRANSCEND FL was to evaluate the efficacy and safety of the CD19-directed chimeric antigen receptor (CAR) T-cell therapy lisocabtagene maraleucel. In this phase 2, single-arm, multicohort study, patients from 30 sites in the USA, Canada, Europe, and Japan with relapsed or refractory MZL who had at least two previous lines of systemic therapy were eligible to receive lisocabtagene maraleucel (100 × 106 CAR+ T cells). Bridging therapy was allowed. The primary endpoint was overall response rate per independent review committee by CT by use of Lugano 2014 criteria (null hypothesis ≤50%). This study is registered with ClinicalTrials.gov, NCT04245839, and is ongoing. Of 77 leukapheresed patients recruited between November 11, 2020, and August 24, 2023, 67 received lisocabtagene maraleucel and 66 were efficacy evaluable. MZL subtypes included nodal (n=32 [48%]), splenic (n=18 [27%]), and extranodal-mucosa-associated lymphoid tissue (n=17 [25%]). Median (IQR) previous lines of systemic therapy was 3 (2-4). Median on-study follow-up was 24·1 months. The primary endpoint was met, with an overall response rate of 95% (n=63; 95% CI, 87·3-99·1; one-sided p<0·0001). All patients experienced a treatment-related adverse event. Grade 3 cytokine release syndrome or neurological events occurred in three (4%) patients each (no grade 4-5 events). 11 (16%) patients had grade ≥3 infections: six (9%) patients during the 90-day treatment-emergent period and seven (10%) during the post-treatment-emergent period. In patients with relapsed or refractory MZL, lisocabtagene maraleucel showed high rates of durable responses. The safety profile was manageable, with no new safety signals. These results support lisocabtagene maraleucel as a new treatment option for patients with relapsed or refractory MZL. Celgene, a Bristol-Myers Squibb Company.
Silent imposter in pneumonic attire: primary pulmonary MALT lymphoma.
Primary pulmonary lymphoma (PPL) is a rare extranodal lymphoma confined to the lungs, accounting for <1% of non-Hodgkin lymphomas. We describe a man in his early 30s presenting with progressive dyspnoea, fever and productive cough, initially treated as pneumonia. Despite multiple antibiotic courses, radiological lesions persisted. Histopathology and immunohistochemistry from transbronchial lung biopsy revealed extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. PET-CT confirmed localised pulmonary involvement, fulfilling criteria for PPL. The patient responded to rituximab-bendamustine chemoimmunotherapy with clinical improvement. This case highlights the importance of considering rare malignancies in non-resolving pulmonary consolidations.
Publicações recentes
The Heart-Brain Connection: Acute Intracranial Pressure Elevation as a Cause of Isolated Bradycardia.
Primary cutaneous marginal zone lymphoma presenting as a violaceous forearm plaque.
Capsule Endoscopy-Guided Diagnosis of Small Bowel Lymphoma Presenting as Protein-Losing Enteropathy: Complementary Role of Peripheral Blood Flow Cytometry.
DNA-based characterization of primary hepatic MALT lymphoma reveals a rare FAT3 missense variant and Hippo pathway activation: a case report and literature review.
Isolated Mental Nerve Neuropathy as the First Clinical Sign of Nodal Marginal Zone Lymphoma: A Diagnostic Challenge.
📚 EuropePMC1.277 artigos no totalmostrando 198
EBV-associated histological transformation of marginal zone lymphomas: two case reports and a literature review.
Virchows Archiv : an international journal of pathologyMarginal Zone Lymphoma: 2026 Update on Diagnosis and Management.
American journal of hematologyDifferent primary thyroid B-cell lymphomas show overlapping mutation profiles, suggesting involvement of a common pathogenic process.
HaematologicaHigh-grade follicular B-cell lymphoma-associated C3 rapidly progressive glomerulonephritis: a rare association.
BMJ case reportsExtranodal Marginal Zone B-cell Lymphoma Presenting as a Painless Buccal Mass in the Masticator Space.
Journal of medical cases[Acquired hemophilia A secondary to marginal zone lymphoma].
Revue medicale de LiegeCutaneous Crystal-Storing Histiocytosis With Marginal Zone Lymphoma. A Case Report With A Striking Clinic Presentation.
Journal of cutaneous pathologySystemic, asymptomatic skeletal muscle infiltration by MALT lymphoma: Defining a 'silent infiltrator' phenotype with 18F-FDG PET/CT.
Leukemia research reportsLow-grade B-cell non-Hodgkin lymphoma in the sternocleidomastoid muscle: a case report and review of literature.
Frontiers in oncologyReal-world single-center analysis of efficacy and safety in newly diagnosed marginal zone lymphoma.
Clinical and experimental medicineCAR T-cell therapy for patients with relapsed or refractory marginal zone lymphoma.
Lancet (London, England)A Meta-analysis Investigating Response Rates with Continuous Bruton Tyrosine Kinase Inhibitor Monotherapies in the Treatment of B Cell Lymphomas.
Oncology and therapyTreatment of disseminated refractory primary cutaneous marginal zone lymphoma with orelabrutinib.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGPatient characteristics and treatment outcomes in marginal zone lymphoma: results of the prospective German MZL registry.
LeukemiaPrognosis in marginal zone lymphoma: a comprehensive review.
HaematologicaComprehensive analysis of mortality risk factors in low-grade B-cell lymphoma.
PloS oneNeoehrlichia mikurensis infection associated with lymphoma or mimicking lymphoma in two cohorts of Swedish patients.
Leukemia & lymphomaField-testing the MALT-IPI in a single center real-world cohort of patients with extranodal marginal zone lymphoma.
Blood advancesBilateral Proptosis Secondary to Extranodal Marginal Zone Lymphoma Mimicking IgG4-Related Disease.
Ophthalmic plastic and reconstructive surgeryExtranodal marginal zone lymphoma presenting as a skin-based breast finding detected on screening mammography.
Radiology case reportsSmall B-cell lymphoma highly suspicious for splenic marginal zone lymphoma with MYD88 L265P mutation and MYC copy-number gain presenting as severe autoimmune hemolytic anemia: a case report with literature review.
Frontiers in immunologyUnusual case of follicular lymphoma of vocal cord.
Virchows Archiv : an international journal of pathologyPrimary Pulmonary Marginal Zone Lymphoma Presenting With Asymptomatic Lung Consolidation.
Case reports in pulmonologyAssessing PET-CT versus conventional CT: a meta-analysis on diagnostic efficacy, prognosis, and post-surgical wound healing in lung mucosal marginal zone lymphoma.
Frontiers in oncologyExtranodal marginal zone lymphoma of mucosa-associated lymphoid tissue with prominent signet ring cell-like plasmacytic differentiation: a diagnostic challenge in small endoscopic biopsy.
PathologyRituximab maintenance in marginal zone lymphoma: Evidence from a real-world single center case series study.
Bulletin du cancer[Updates in marginal zone lymphomas].
Annales de pathologieDiagnostic Challenge of Extranodal Marginal Zone Lymphoma of Extraocular Muscles: A Case Report.
Reports (MDPI)Lesion-level dual-tracer PET biomarkers predict prognosis in multiple myeloma treated with CXCR4-directed radiopharmaceutical therapy.
European journal of nuclear medicine and molecular imagingThree Years of Idiopathic Chylous Ascites: Splenic Marginal Zone Lymphoma Behind the Mask.
CureusWhen Benign Mimics Malignancy: Parapharyngeal Oncocytoma in a Patient With Marginal Zone Lymphoma.
Clinical case reportsLisocabtagene maraleucel in patients with relapsed or refractory marginal zone lymphoma (TRANSCEND FL): primary analysis results from the global, multicohort, single-arm, phase 2 study.
Lancet (London, England)Paraneoplastic reactive perforating collagenosis in setting of Hodgkin lymphoma and prior marginal zone lymphoma.
JAAD case reportsSurvival outcomes of eyelid lymphoma: a SEER database analysis.
Orbit (Amsterdam, Netherlands)Silent imposter in pneumonic attire: primary pulmonary MALT lymphoma.
BMJ case reportsAtraumatic Splenic Rupture as the Initial Manifestation of Splenic Marginal Zone Lymphoma: A Case Report.
CureusPrognostic Factors and Survival Outcomes in Marginal Zone Lymphoma: Real-Life Experience.
Turkish journal of haematology : official journal of Turkish Society of HaematologyIndolent primary cutaneous B-cell lymphomas resemble persistent antigen reactions without signs of dedifferentiation.
Nature communicationsClinical characterization and targeted genetic mutation profiling of autoimmune disease-associated B-cell lymphoma.
Discover oncology[Diagnostic challenges in pediatric lymphomas].
Pathologie (Heidelberg, Germany)IgG4-Positive Ocular Adnexal Extranodal Marginal Zone Lymphoma as an Aggressive Clinical Subtype: Evidence From a 377-Patient Cohort.
American journal of ophthalmologyA case of marginal zone lymphoma presenting with paraneoplastic vasculitis and pulmonary infiltration.
Respiratory medicine case reportsDiscordant lymphoma consisting of splenic marginal zone lymphoma and diffuse large B-cell lymphoma in a hepatitis B virus carrier suggests virus-associated lymphomagenesis.
Journal of clinical and experimental hematopathology : JCEHBTK Inhibition in Hematology: From CLL/SLL to Emerging Applications Across B-Cell and Immune Disorders.
BiomoleculesMucosa-associated lymphoid tissue lymphoma with monoclonal T-cell expansion.
Virchows Archiv : an international journal of pathologyEpigenetic dysregulation of B‑cells in autoimmune diseases and lymphomas (Review).
International journal of molecular medicineMarginal zone lymphoma presenting with chylous ascites: A rare clinical manifestation and successful treatment with orelabrutinib-rituximab.
SAGE open medical case reportsPirtobrutinib, a Highly Selective, Non-covalent (Reversible) BTKi in R/R Marginal Zone Lymphoma: Phase 1/2 BRUIN Study.
Blood advancesClinical and Diagnostic Challenges in Primary Splenic Lymphomas: When is Splenectomy Necessary to Differentiate SMZL from DLBCL?
European journal of case reports in internal medicineZanubrutinib for the treatment of cutaneous relapse of systemic marginal zone lymphoma: A case report.
JAAD case reportsRare and varied presentations of plasmablastic lymphoma: A case series.
SAGE open medical case reportsPulmonary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in a common variable immunodeficiency patient.
Medicina clinicaLow-Grade B-cell Lymphoma in Primary Sjögren's Syndrome: A Case Report.
CureusNodal Marginal Zone Lymphoma with Prominent Expansion of PD-1+ T-Follicular Helper Cells: A Persistent Diagnostic Challenge with a Heterogeneous Mutational Architecture.
International journal of molecular sciencesAcetanilide herbicide exposure and lymphoid malignancy risk in the French AGRiculture and CANcer cohort.
Environment internationalIntroduction to a review series on marginal zone lymphoma: reclaiming the afterthought.
BloodNon-gastric mucosa-associated lymphoid tissue lymphomas: a narrative review of pathogenesis, diagnosis, and treatment strategies.
Annals of translational medicineRosai-Dorfman disease mimicking testicular malignancy in a patient with marginal zone lymphoma of the lung.
BMJ case reportsClonal Hematopoiesis and Lymphoma-Associated Mutations in Hematopoietic Progenitors in B-Cell Non-Hodgkin Lymphoma.
BloodEfficacy and Safety of Orelabrutinib for Previously Treated Marginal Zone Lymphoma.
Journal of hematologyDiagnostic challenges and management strategies of pulmonary mucosa-associated lymphoid tissue lymphoma: a case report and literature review.
Frontiers in medicinePrimary Breast MALT Lymphoma: Clinical Features and Outcomes From a Single-Institution Experience at Mayo Clinic.
American journal of hematologyMeta-analysis of comparing CAR-T and bispecific antibody therapy in relapsed/refractory indolent B-cell non-Hodgkin's lymphomas.
Journal of translational medicineSecondary cutaneous marginal zone lymphoma arising in association with a Mohs micrographic surgery graft site.
JAAD case reportsMucosal-Associated Lymphoid Tissue Lymphoma in Southeast Asia: A 15-Year Retrospective Multicenter Study.
Hematology reportsEfficacy and Safety of Capivasertib (AZD5363), a Potent, Oral Pan-AKT Inhibitor, in Patients with Relapsed or Refractory B-cell Non-Hodgkin Lymphoma (CAPITAL).
Clinical cancer research : an official journal of the American Association for Cancer ResearchExpression profiling reveals coordinated dysregulation of autophagy-associated proteins in marginal zone lymphoma and therapeutic implications.
Oncology lettersA Phase II Study of Bendamustine Plus Rituximab in Patients with Relapsed or Progressive Marginal Zone Lymphoma: KCSG LY14-09.
Cancer research and treatmentVenetoclax and ibrutinib induces durable clinical responses in marginal zone lymphoma.
Blood advancesReduced-Dose Hypofractionated Radiation Therapy (3 Gy × 3 Fractions) for Indolent Non-Hodgkin's lymphoma (POSEIDON): A Multisite Phase 2 Randomized Trial Protocol.
Advances in radiation oncologyEmerging Epidemiological Trends in Primary Cutaneous Lymphomas: A Population-Based Analysis.
Clinical and experimental dermatology[The guideline for the diagnosis and treatment of marginal zone lymphoma in China (2025)].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiNodal Marginal Zone Lymphoma with Eosinophilic Dermatosis of Haematologic Malignancy: A Case Report.
Acta dermato-venereologicaExtranodal MALT lymphoma masquerading as a cavernous hemangioma: A diagnostic and surgical challenge.
Radiology case reportsComparative effectiveness of immunotherapy alone or with chemotherapy as first-line treatment for marginal zone lymphoma.
HaematologicaSite-specific prognosis and temporal trends in extranodal marginal zone lymphoma patients in the United States.
Blood cancer journalUltra low dose radiotherapy for primary ocular adnexal marginal zone lymphoma.
Orbit (Amsterdam, Netherlands)A Rare Case of Subglottic Malignant Lymphoma Requiring Emergency Tracheostomy.
CureusIgG4 plasma cells in hematopathology: beyond IgG4-related disease.
Journal of hematopathologyThe diagnostic utility and frequency of CD56 expression in plasma cell myeloma.
Annals of diagnostic pathologyLight-chain-restricted hematogones are a potential diagnostic pitfall for B-cell lymphoma.
Indian journal of pathology & microbiologyPediatric-Type Follicular Lymphoma of the Conjunctiva: Shared Histologic and Molecular Features With the Nodal Counterpart.
The American journal of surgical pathologyConjunctival Extranodal Marginal Zone Lymphoma Presenting as Entropion.
Acta medica portuguesaRituximab-Induced Acute Coronary Syndrome.
CureusTransformation of Classical Hodgkin Lymphoma Into Non-Hodgkin Lymphoma: Three Case Reports From the West Bank.
Case reports in oncological medicineChemokine receptor-4-targeted imaging with [68Ga]Ga-Pentixafor PET: unveiling its diagnostic and prognostic potential across cancers: a systematic review.
Nuclear medicine communicationsSynchronous Primary Pleural Mucosa-Associated Lymphoid Tissue Lymphoma and Pulmonary Squamous Cell Carcinoma in a Patient With Asbestos Exposure: A Case Report.
CureusPeripheral blood involvement in extra-nodal marginal zone lymphoma.
British journal of haematology[Mixed type II cryoglobulinemiaassociated glomerulonephritis revealing an extragastric marginal zone lymphoma in the absence of viral infection: A rare case report from Bourges Hospital].
Nephrologie & therapeutiquePrimary Cutaneous B-Cell Lymphomas: An Updated Portrait of Classification, Biology, and Clinical Management.
European journal of haematology[Primary cutaneous B-cell lymphomas-an overview of established and novel aspects].
Dermatologie (Heidelberg, Germany)BCL-2 inhibition in Waldenström macroglobulinaemia and marginal zone lymphoma.
British journal of haematologyCD180 as a Robust Immunophenotypic Marker for Differentiating Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia From Marginal Zone Lymphoma.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncFive-Year Follow-Up Analysis of ZUMA-5: Axicabtagene Ciloleucel in Relapsed/Refractory Indolent Non-Hodgkin Lymphoma.
Journal of clinical oncology : official journal of the American Society of Clinical OncologyTwo B or not two B; the question of bendamustine dosing in low grade lymphoma.
Pathology oncology research : PORRoutine interventions failed to provide survival benefits for early-stage major salivary gland marginal zone lymphoma in a population-based analysis.
Annals of hematologyCase report: Histiocytic sarcoma as transdifferentiation of a marginal zone lymphoma-a case presentation based on post mortem examination and review of the literature.
Frontiers in oncologyThe biology of marginal zone lymphoma subtypes: challenge and relevance of classification.
BloodA Rare Case of Thymic Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma.
CureusSurvival Machine Learning Methods Improve Prediction of Histologic Transformation in Follicular and Marginal Zone Lymphomas.
CancersClinical and molecular features of primary spinal epidural lymphomas.
Annals of hematologyA hidden clue behind angioedema in an elderly patient.
Oxford medical case reportsPrimary Cutaneous B-cell Lymphoma Presenting in an Adolescent Male: A Case Report.
CureusDrug development in MZL: caring for the forgotten child.
BloodNodal Marginal Zone B-Cell Lymphoma of a Single Lymph Node in the Adult Neck:Report of One Case.
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae SinicaeTargeted Radiotherapy in Primary Cutaneous Lymphomas: Precision, Efficacy, and Evolving Strategies.
Cancers[The Relationship between Ig Class Switch Recombination and MMR Protein, Microsatellite Phenotype in Extranodal Marginal Zone Lymphoma of Mucosa-associated Lymphoid Tissue].
Zhongguo shi yan xue ye xue za zhiUnexpected Diagnosis of Non-Hodgkin Lymphoma After Liver Transplant: A Case Report of Suspected Neuroendocrine Tumor Recurrence Managed with Robotic Distal Pancreatectomy and Splenectomy.
Experimental and clinical transplantation : official journal of the Middle East Society for Organ TransplantationZosteriform Primary Cutaneous Marginal Zone Lymphoma-A Case Report.
The Australasian journal of dermatologyEpidermotropic Methotrexate-Associated Diffuse Large B-Cell Lymphoma Presenting With Disseminated Papular Eruptions.
The Journal of dermatology[Primary lymphomas in urinary bladder: a clinicopathological analysis of 23 cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyGenetically determined body mass index is associated with diffuse large B-cell lymphoma in polygenic and Mendelian randomization analyses.
International journal of cancerCase Report: Diagnosis of splenic marginal zone lymphoma by ultrasound and contrast- enhanced ultrasound.
Frontiers in oncologyPulmonary mucosa-associated lymphoid tissue lymphoma (MALToma): Clinico-radiologic features, diagnosis, and outcomes.
Respiratory medicineBehavioral investigation of reactive and neoplastic lymphocytes in human lymph nodes in 4D.
PloS oneCutaneous AL-Type Amyloidomas as a Variant of Primary Cutaneous Marginal Zone Lymphoma: A Series of 30 Patients.
The American Journal of dermatopathologyClinical Patterns and Prognostic Outcomes of Asian Ocular Adnexal Marginal Zone Lymphoma.
Journal of hematologyCase Report: Two cases of primary pulmonary endobronchial extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue localized in the trachea and bronchus.
Frontiers in oncologySuccessful Treatment of Acquired von Willebrand Syndrome in a Rare Case of Nodal Marginal Zone Lymphoma With Rituximab Therapy: A Case Report.
CureusSplenic marginal zone lymphoma associated with chronic inflammatory demyelinating polyradiculoneuropathy: a case report.
Journal of medicine and lifePrognostic Significance of Paraproteinemia in Marginal Zone Lymphoma.
Turkish journal of haematology : official journal of Turkish Society of HaematologyPrimary Hepatic Mucosa-Associated B-Cell Lymphoma in a Patient with Primary Sclerosing Cholangitis-A Case Ultimately Requiring Liver Transplantation.
Diagnostics (Basel, Switzerland)CXCR-4 -directed PET/CT examination in a case of marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma).
RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der NuklearmedizinShared genetic susceptibility between idiopathic inflammatory myopathies and common B cell lymphoma subtypes found primarily in the human leucocyte antigen region.
RMD openCrystal-storing histiocytosis associated with MALT Lymphoma: a case report and literature review.
Annals of medicine and surgery (2012)Malignant Hematolymphoid Plasmacytic Effusions: A Cytopathologic Study of Six Cases.
Diagnostic cytopathologyAtrial fibrillation following bendamustine in three lymphoma patients.
Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy PractitionersDifferential diagnosis of multiple myeloma.
Seminars in diagnostic pathologyNuclear Budding in Peripheral Blood Smear of Nodal Marginal Zone Lymphoma.
International journal of laboratory hematologyPolygenic Risk, Agent Orange Exposure, and Lymphoid Neoplasms in the Veterans Affairs Million Veteran Program.
JAMA network openOrbital atypical lymphocytic panniculitis preceding conjunctival marginal zone lymphoma in the same patient with undifferentiated connective tissue disease: a case-based review.
Rheumatology internationalCardiac amyloidosis associated with marginal zone lymphoma.
European heart journal. Case reportsEuropean Organisation for Research and Treatment of Cancer, United States Cutaneous Lymphoma Consortium and International Society for Cutaneous Lymphomas consensus recommendations for management and treatment of cutaneous lymphoproliferative disorders.
The British journal of dermatologyIdentifying high-risk patients with MALT lymphoma undergoing first-line therapy for disease progression.
Expert review of hematologyCrystal-Storing Histiocytosis of the Stomach: An Unusual Clinical Context of a Rare Entity.
International journal of surgical pathology[Male genital system lymphoma: Clinicopathological analysis of 80 cases].
Zhonghua nan ke xue = National journal of andrologyAutoimmune Hemolytic Anemia as an Initial Manifestation of Splenic Marginal Zone Lymphoma.
CureusSalvage Irradiation for Ocular Adnexal Mucosa Associated Lymphoid Tissue Lymphoma Refractory to Chlamydia psittaci Eradication.
Advances in radiation oncologySuspicious Atypical B-Cell Infiltrates-Significance of Work-up in Urinary and Male Genital Organs.
International journal of surgical pathology68Ga-pentixafor for PET imaging of chemokine receptor 4 expression in lymphoproliferative diseases and solid tumors.
Hellenic journal of nuclear medicineUnusual Clinical Presentation of B-Cell Prolymphocytic Leukemia Cases as Splenic Marginal Zone Lymphoma Effectively Treated With Rituximab Monotherapy.
Anticancer researchBlastoid-appearing lymphocytes in a case of leukemic marginal zone lymphoma.
Annales de biologie cliniqueAcquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature.
Journal of primary care & community healthHistiocytic Sarcoma Arising in a Background of Nodular Lymphocyte Predominant Hodgkin Lymphoma.
Case reports in hematologyBronchial artery embolization for haemoptysis in patients with lymphoma and leukaemia.
BJR case reportsEffects of a cancer-associated mutation and multiple serine phosphorylation on poly(ADP-ribose) polymerase 2.
Biophysical journalGenomic characterization of clonal B-cell lymphocytosis of marginal zone origin with MYC rearrangements.
Virchows Archiv : an international journal of pathologyPrımary Esophageal Extranodal Margınal Zone Lymphoma: A Rare Tumor : Primary Esophageal Lymphoma in a Patient Presenting as Dysphagia.
Digestive diseases and sciencesThe Role of Artificial Intelligence and Radiomics in the Management of Lymphomas by PET/CT: The Clairvoyance in Clinic.
Cancer management and researchFrom the archives of MD Anderson Cancer Center: Small lymphocytic lymphoma/chronic lymphocytic leukemia diagnosed in Warthin tumor.
Annals of diagnostic pathologyIndolent pediatric lymphomas/lymphoproliferative disorders: a report of the 2024 EA4HP/SH lymphoma workshop.
Virchows Archiv : an international journal of pathologyObservations on seasonal occurrence in lymphomas with proven or presumed infectious etiology.
Frontiers in oncologyParaneoplastic Erythroderma: A Rare, but Important Cause of Mixed Pattern Dermatitis.
Journal of cutaneous pathologyReal-World Efficacy and Safety of the Combination of Rituximab, Lenalidomide, and Ibrutinib in Patients with Relapsed and/or Refractory Non-Hodgkin Lymphoma.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionIntegrated histomolecular approach to discriminate primary cutaneous follicle centre and marginal zone lymphoma of the scalp.
PathologySevere acute allograft rejection 22 years after liver transplantation.
Canadian liver journalAutonomous B-cell Receptor Signaling in Chronic Lymphocytic Leukemia.
Hematology/oncology clinics of North AmericaThe treatment of marginal zone lymphoma.
BloodKLF2 double variants (C274Y and H210N) in a splenic B-cell lymphoma/leukemia: diagnostic insights into splenic marginal zone lymphoma and mechanistic implications.
Leukemia & lymphomaIL-16 production is a mechanism of resistance to BTK inhibitors and R-CHOP in lymphomas.
bioRxiv : the preprint server for biologyPrimary cutaneous marginal zone lymphoma in a patient with multiple sclerosis under fingolimod therapy.
Dermatology reportsExploring the Genetic Landscape of Primary Marginal Zone Lymphoma of the Urinary Bladder.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncNodal Marginal Zone Lymphoma with Simultaneous Transformation at Diagnosis and Pleural Effusion as the Initial Presentation: A Case Report.
Acta medica LituanicaLow-Grade B-Cell Neurolymphomatosis: A Case Series and Diagnostic Insight.
World neurosurgeryAcquired angioedema and rituximab-induced acute thrombocytopenia in splenic marginal zone lymphoma.
Acta oncologica (Stockholm, Sweden)Exploring the boundaries between neoplastic and reactive lymphoproliferations: lymphoid neoplasms with indolent behavior and clonal lymphoproliferations-a report of the 2024 EA4HP/SH lymphoma workshop.
Virchows Archiv : an international journal of pathologyMultifocal Cutaneous Amyloidosis Mimicking Scleroderma Associated With Primary Cutaneous Marginal Zone Lymphoma.
The Journal of dermatologyIRTA1 expression in extranodal marginal zone (MALT) lymphoma is variable across anatomic sites.
American journal of clinical pathologyPredictors of survival in gastric mucosa-associated lymphoid tissue lymphoma: An updated surveillance, epidemiology, and end results-based analysis of age and gender disparities.
World journal of clinical oncologyRecurrent Composite Lymphoma in an Elderly Man: A Case of Marginal Zone Lymphoma With Classic Hodgkin Features Treated With Brentuximab and Immunotherapy.
CureusSplenic Diffuse Red Pulp Small B-cell Lymphoma: A Rare Diagnosis in a Patient With Incidental Abnormal Lymphocytosis.
CureusMarginal zone lymphoma masquerading as phymatous acne rosacea: a case study.
Skin health and diseaseRefractory marginal zone lymphoma uncovers activated phosphoinositide 3-kinase delta syndrome type 1 (APDS1).
The journal of allergy and clinical immunology. GlobalSOHO State of the Art Updates and Next Questions | Current and Emerging Novel Treatments for Marginal Zone Lymphoma.
Clinical lymphoma, myeloma & leukemiaA case of intestinal MALT lymphoma characterized by diffuse capillary hyperplasia nodules with hemorrhage.
Revista espanola de enfermedades digestivasCD5 expression in marginal zone lymphoma does not predict inferior outcome and has similarities to indolent lymphomas.
Blood neoplasiaResistance Mechanism for Zanubrutinib in Marginal Zone Lymphoma.
Journal of the National Comprehensive Cancer Network : JNCCN[Angioedema Due to Acquired C1 Inhibitor Deficiency as a Clinical Manifestation of Marginal Zone Lymphoma: A Case Report].
Revista medica de ChileA Unique Case Presentation of Synchronous Early-Stage Invasive Lobular Carcinoma of the Breast and Marginal Zone Lymphoma of the Ipsilateral Breast.
CureusThe anti-CD47 antibody magrolimab with obinutuzumab and venetoclax in relapsed or refractory indolent B-cell lymphomas.
British journal of haematologyMarginal Zone Lymphoma: Treatment Update With a Focus on Systemic Approaches.
Hematological oncologyIGH/IGK gene rearrangement in the diagnosis of B-cell non-Hodgkin lymphoma: experience from three centers.
Annals of hematologyPerspectives on T-cell engaging therapies in relapsed/refractory indolent non-Hodgkin lymphoma.
Current opinion in oncology[Radiotherapy of non-Hodgkin lymphoma-discussion of modern treatment concepts and innovations].
Radiologie (Heidelberg, Germany)[Clinical and cytological characteristics of primary thyroid lymphoma: a fine needle aspiration study of 10 cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyConcurrent chronic follicular conjunctivitis and conjunctival MALT lymphoma potentially caused by Chlamydia pneumoniae.
IDCasesExtranodal Marginal Zone Lymphoma Presenting as Acute Kidney Injury due to Cast Nephropathy: A Case Report.
Canadian journal of kidney health and diseaseA rare case of co-occurrence of lupus nephritis and lymphoma.
Indian journal of pathology & microbiologyMarginal Zone Lymphoma With Extensive Skeletal Involvement and Hypercalcemia: A Rare Case With a Systematic Review of the Literature.
CureusAntigen selection reflected in the subclonal architecture of the B-cell receptor immunoglobulin gene repertoire in splenic marginal zone lymphoma.
HemaSphereA Case of Nodal Marginal Zone Lymphoma Presenting as Left Leg Swelling.
CureusPhenotypic Profile of Waldenström Macroglobulinaemia B-Cells: Establishment of a Diagnosis Scoring System and Clinico-Biological Correlations.
Journal of cellular and molecular medicinePET/CT in the Staging and Treatment Response Assessment of Patients With Extranodal Marginal Zone Lymphoma.
American journal of hematologyAcquired angio-oedema as a mimicker of drug allergy.
BMJ case reportsMarginal zone lymphoma with anti-factor H IgM and atypical hemolytic uremic syndrome successfully treated with odronextamab.
HaematologicaImpact of diagnosis to treatment interval on outcomes in patients with newly diagnosed marginal zone lymphoma - a US multisite study.
Experimental hematology & oncologyMolecular profiling of primary renal marginal zone lymphoma reveals overlapping features with extranodal marginal zone lymphoma.
Blood advancesA case of pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma in a patient with a history of idiopathic lymphocytic interstitial pneumonia (iLIP).
General thoracic and cardiovascular surgery casesAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Linfoma da zona marginal.
É de uma associação que acompanha esta doença? Fale com a gente →
Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Linfoma da zona marginal e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Não há padrão de herança familiar estabelecido nos catálogos genéticos para o linfoma da zona marginal. As mutações associadas aos genes MALT1, BCL10 e BIRC3 costumam ocorrer nas células do tumor durante a idade adulta.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (s.d.). Linfoma da zona marginal. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/linfoma-da-zona-marginal
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Linfoma da zona marginal
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Medicamentos aprovados FDA
- fonte: FDA OpenFDA
- Ensaios clínicos
- fonte: ClinicalTrials.gov
