Introdução
O que você precisa saber de cara
Linfoma intraocular é uma forma maligna rara de câncer ocular. O linfoma intraocular pode afetar o olho secundariamente a partir de uma metástase de um tumor não-ocular ou pode surgir primariamente dentro do olho. O LIO P é um subtipo de linfoma primário do sistema nervoso central (LPSNC). Os LPSNC são mais comumente um subtipo imunohistoquímico de linfoma difuso de grandes células B não-Hodgkin, de acordo com a classificação de linfomas da Organização Mundial da Saúde (OMS). Os sintomas mais comuns do LIO P incluem visão turva ou diminuída devido a células tumorais no vítreo. A maioria dos casos de LIO P evoluem para envolvimento do sistema nervoso central (LPSNC), enquanto apenas 20% dos LPSNC levam a envolvimento intraocular (LIO P).
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
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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 — Primary vitreoretinal large B-cell lymphoma
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Publicações mais relevantes
Real world outcomes of intravitreal and systemic therapy in primary and secondary vitreoretinal lymphoma.
Vitreoretinal large B-cell lymphoma (VR-LBCL) is a rare hematologic malignancy. It is classified as primary (PVR-LBCL) or secondary (SVR-LBCL) based on the initial site of manifestation. Owing to limited prospective data and absent standardized guidelines, treatment remains challenging. This dual-center retrospective study aimed to evaluate outcomes of methotrexate (MTX) intravitreal (itv.), Rituximab itv., or combinatorial itv. therapy (R-MTX) and assess the impact of additional systemic immunochemotherapy. Among 65 patients (median age 72 years) included, 55.4% (n = 36) had PVR-LBCL. The median time to diagnosis was 31 days (1–2805). Over a median follow-up of 23.2 months, 35 patients relapsed. MTX itv. showed a trend toward better ocular relapse-free-survival than Rituximab itv. (P = 0.07). Intriguingly, patients receiving R-MTX itv. experienced no relapses throughout follow-up. In general, addition of systemic therapy was associated with significantly longer relapse-free-survival compared to itv. monotherapy (P = 0.05). Keratopathy and elevated intraocular pressure were common side effects with MTX itv. and Rituximab itv., respectively. Despite compelling findings and being one of the largest cohorts published to date, the heterogeneity of the patient population and small subgroups limit direct clinical translation. Nonetheless, this study provides a strong foundation for the design of future clinical trials. The online version contains supplementary material available at 10.1038/s41598-026-37804-4.
Immune-Mediated Macular Edema After Intravitreal Methotrexate in Primary Vitreoretinal Lymphoma.
To report a case of cystoid macular edema in a patient with primary vitreoretinal lymphoma (PVRL) following intravitreal methotrexate (MTX) and lymphoma remission. Case report. A 76-year-old man was diagnosed with PVRL after a 9-month history of bilateral floaters and progressive blurred vision. Vitreous biopsy revealed CD20-positive large B cells, a MYD88 (L265P) mutation, and interleukin IL-10/IL-6 ratio of 1.76 at diagnosis. Systemic involvement was initially excluded but metachronous brain lesions emerged, prompting systemic chemotherapy.Macular edema developed after 2 and 6 weeks of MTX in the left and right eyes, respectively, coinciding with inversion of the IL-10/IL-6 ratio to 0.01. Edema was refractory to topical therapy and required intravitreal dexamethasone and aflibercept. The simultaneous inversion of the IL-10/IL-6 ratio with edema onset suggests a cytokine shift unmasking an inflammatory response previously suppressed by malignant lymphoproliferation. This pattern supports an immune reconstitution inflammatory syndrome-like mechanism as the most likely cause.
Intraocular Lymphoma: A Review.
Intraocular lymphoma (IOL) is a rare form of non-Hodgkin lymphoma that primarily presents in two distinct types. The first type, known as primary intraocular lymphoma (PIOL), is mainly recognized as a subtype of primary central nervous system lymphoma (PCNSL). Recent classifications have emphasized the primary ocular sites affected, with vitreoretinal lymphoma emerging as the most common variant linked to PCNSL. Despite its rarity, the incidence of PIOL is rising among both immunocompromised and immunocompetent populations. Most cases of PIOL are identified as diffuse large B-cell lymphoma, although rare T-cell variants have also been reported. Secondary intraocular lymphoma (SIOL) originates from metastatic spread of non-CNS lymphomas to the ocular structures, including the retina, uvea, vitreous body, Bruch's membrane, and optic nerve. Diagnosis of IOL is challenging for ophthalmologists and pathologists, as it can easily mimic other ocular conditions. Advancements in laboratory diagnostics, such as immunocytochemistry, flow cytometry, and the evaluation of interleukin ratios (specifically IL-10:IL-6 > 1), along with polymerase chain reaction (PCR) amplification for clonality, have enhanced diagnostic accuracy. Multimodal imaging approaches and molecular analyses can serve as valuable indicators of visual prognosis, recurrence rates, and the likelihood of progression to central nervous system involvement. Given that misdiagnosis or delayed diagnosis can result in serious treatment delays and potentially life-threatening outcomes for patients with IOL, this review seeks to provide a comprehensive understanding of the clinical manifestations of IOL and the diagnostic methods employed.
Clinicopathological Significance of Tumor-Infiltrating T Lymphocytes and Macrophages in Primary Large B-Cell Lymphoma of Immune-Privileged Sites.
Immune-privileged large B-cell lymphomas (IP-LBCLs), comprising primary central nervous system lymphoma (PCNS-LBCL), primary vitreoretinal lymphoma (PVR-LBCL), and primary testicular lymphoma (PT-LBCL), originate in sites with limited immune surveillance. Owing to their rarity, the prognostic implications of the tumor microenvironment in IP-LBCLs remain unclear, warranting further investigation. This study evaluated 109 IP-LBCL cases (PCNS-LBCL, n=87; PT-LBCL, n=22; six cases of PVR-LBCL excluded) using multiplex immunohistochemistry on tissue microarrays, along with clinicopathological analysis. Immune cell infiltration, tumor major histocompatibility complex (MHC) class I, and programmed death ligand-1 (PD-L1) expression, and their associations with clinical outcomes, were evaluated. PT-LBCL demonstrated higher infiltration of all tumor-infiltrating T lymphocyte (TIL) subsets than PCNS-LBCL (all p<0.05). Elevated CD4⁺ and CD8⁺ T-cell levels correlated with prolonged progression-free survival (PFS) (both p<0.05). M1 macrophage infiltration was associated with improved PFS (p=0.005) and independently predicted a favorable prognosis (hazard ratio = 0.49, p=0.041). Loss of MHC class I expression was more frequent in PT-LBCL than in PCNS-LBCL (77.3% vs. 9.2%; p<0.001). TIL infiltration predicted improved PFS only when the tumor MHC class I was preserved. Moreover, programmed death protein-1 (PD-1)⁺ TILs and tumor PD-L1 expression were associated with prognosis in conjunction with various clinicopathological variables. These findings highlight the favorable prognostic role of TILs and M1 macrophages, and underscore the complex immune-tumor interactions in IP-LBCLs, despite their origin in immune-privileged sites.
Whole-Eye Radiation for the Local Control of Choroidal Lymphoma in Primary Central Nervous System Lymphoma: A 14-Year Case Study.
Involved-site radiation therapy is effective for curative and palliative treatments of cancers, including lymphoma. This case study describes the use of whole-eye radiation for primary intraocular lymphoma occurring during primary central nervous system lymphoma. The patient, a 68-year-old man, developed personality changes and apathy two weeks after cataract surgery combined with vitrectomy for vitreous opacity in the left eye. Magnetic resonance imaging revealed a mass lesion in the left frontal lobe, and biopsy by craniotomy confirmed diffuse large B-cell lymphoma. He underwent chemotherapy using rituximab combined with high-dose methotrexate and high-dose cytarabine in association with intrathecal methotrexate and cytarabine injections, leading to complete remission. At age 75, he noticed forgetfulness, and fluorodeoxyglucose positron emission tomography and magnetic resonance imaging revealed a relapse of lymphoma in the splenium of the corpus callosum. He underwent chemotherapy using rituximab combined with high-dose methotrexate, followed by monthly rituximab monotherapy for one year and then rituximab monotherapy every two months for one year. He maintained complete remission with no treatment until age 78, when he developed subretinal choroidal lesions in the left eye and underwent whole-eye radiation at 40 Gy. One year later, he developed subretinal choroidal lesions in the right eye and underwent whole-eye radiation at 40 Gy. At age 81, he had lower limb weakness with disorientation. Magnetic resonance imaging showed a relapse of lymphoma in the right frontal to temporal lobe. The brain lesions showed a marked response to four weeks of oral tirabrutinib as a salvage therapy, but the lesions regrew, and the patient died seven months later. Throughout the treatment, he maintained a visual acuity of 0.7 (decimal scale) in both eyes. In conclusion, whole-eye radiation should be considered as a treatment option for the local control of active intraocular lymphoma, especially choroidal lesions, for patients with primary central nervous system lymphoma with no active brain lesions and without systemic treatment.
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The global impact of imiglucerase therapy in children with Gaucher disease types 1 and 3: a real-world analysis from the International Collaborative Gaucher Group Gaucher Registry.
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Real world outcomes of intravitreal and systemic therapy in primary and secondary vitreoretinal lymphoma.
Scientific reportsIntraocular Lymphoma: A Review.
Journal of ophthalmic & vision researchImmune-Mediated Macular Edema After Intravitreal Methotrexate in Primary Vitreoretinal Lymphoma.
Ocular immunology and inflammationClinicopathological Significance of Tumor-Infiltrating T Lymphocytes and Macrophages in Primary Large B-Cell Lymphoma of Immune-Privileged Sites.
Cancer research and treatmentWhole-Eye Radiation for the Local Control of Choroidal Lymphoma in Primary Central Nervous System Lymphoma: A 14-Year Case Study.
CureusA Novel Prognostic Score for Disease Progression and Mortality in Patients With Newly Diagnosed Primary Large B-Cell Lymphoma of Immune-Privileged Sites.
Hematological oncologyMolecular diagnosis of vitreoretinal lymphoma.
Taiwan journal of ophthalmologyOptimal utilization of paucicellular vitreous sample for diagnosis of primary vitreoretinal lymphoma.
Journal of the American Society of CytopathologyA Natural Course From Primary Intraocular Lymphoma to Brain Lymphoma in Four Years According to Patient's Choice.
CureusIn the era of targeted therapy and immunotherapy: advances in the treatment of large B-cell lymphoma of immune-privileged sites.
Frontiers in immunologyA case of primary intraocular B-cell lymphoma masquerading alongside varicella-zoster virus retinitis.
American journal of ophthalmology case reportsA glaucoma drainage implant functioning as a sanctuary site for vitreoretinal lymphoma.
American journal of ophthalmology case reportsAdvances in primary large B-cell lymphoma of immune-privileged sites.
Frontiers in immunologyPrimary Cerebral Lymphoma With Isolated Vitreoretinal and Cerebral Recurrences Without Meningeosis: A Case Report.
CureusEndoretinal biopsy outcomes in suspected vitreoretinal lymphoma after inconclusive vitreous cytology.
Journal francais d'ophtalmologieMycophenolate Mofetil-Induced Lymphoproliferative Disorder in a Young Adult With Chronic Posterior Uveitis.
Journal of vitreoretinal diseasesVitreoretinal large B- cell lymphoma (VR- LBCL): Clinical and pathological features and treatment outcomes.
Pathology, research and practicePrimary diffuse large B-cell lymphoma of the central nervous system identified with CSF biomarkers.
BMC neurologyTransient Bacillary Layer Detachment During the Disease Course of Primary Vitreoretinal Lymphoma.
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Survey of ophthalmologyHyper-N-glycosylated SEL1L3 as auto-antigenic B-cell receptor target of primary vitreoretinal lymphomas.
Scientific reportsDiagnosis of primary vitreoretinal lymphoma masquerading infectious retinitis by retinal biopsy.
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Ocular immunology and inflammationPrimary Autologous Stem Cell Transplantation for Unilateral Primary Central Nervous System Lymphoma-Ophthalmic Variant (Primary Vitreoretinal Lymphoma).
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Journal of hematology & oncology[Lymphoma of the eye and its adnexa : Modern pathological diagnostics and systemic treatment].
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BMC ophthalmologyOptical coherence tomography manifestations of primary vitreoretinal lymphoma.
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Retina (Philadelphia, Pa.)Advances in Primary Central Nervous System Lymphoma.
Current oncology reports[Intraocular lymphoma associated with primary malignant lymphoma of the central nervous system: Seven-year experience of a tertiary center].
Journal francais d'ophtalmologiePrimary vitreoretinal lymphoma.
Indian journal of ophthalmologyHigh frequency of MYD88 mutations in vitreoretinal B-cell lymphoma: a valuable tool to improve diagnostic yield of vitreous aspirates.
BloodCorrelation between measurement of IL-10 and IL-6 in paired aqueous humour and vitreous fluid in primary vitreoretinal lymphoma.
Acta ophthalmologica[Diagnostics and treatment of primary vitreoretinal lymphoma].
Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen GesellschaftAssociações
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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.
- Real world outcomes of intravitreal and systemic therapy in primary and secondary vitreoretinal lymphoma.
- Immune-Mediated Macular Edema After Intravitreal Methotrexate in Primary Vitreoretinal Lymphoma.
- Intraocular Lymphoma: A Review.
- Clinicopathological Significance of Tumor-Infiltrating T Lymphocytes and Macrophages in Primary Large B-Cell Lymphoma of Immune-Privileged Sites.
- Whole-Eye Radiation for the Local Control of Choroidal Lymphoma in Primary Central Nervous System Lymphoma: A 14-Year Case Study.
- Mast cell mediators in hereditary angioedema.
- Prenatal Molecular Diagnosis of COL2A1-Associated Stickler Syndrome: Genotype-Phenotype Correlation in a Resource-Limited Healthcare Setting.
- Platelet gene signatures detecting pulmonary artery stenosis in patients with pulmonary hypertension.
- The global impact of imiglucerase therapy in children with Gaucher disease types 1 and 3: a real-world analysis from the International Collaborative Gaucher Group Gaucher Registry.
- Monogenic lupus with SLC7A7 mutations: a retrospective study from a Chinese center.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:695631(Orphanet)
- MONDO:0979292(MONDO)
- Busca completa no PubMed(PubMed)
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
