ArticleBlood advances2020
Primary vitreoretinal lymphomas display a remarkably restricted immunoglobulin gene repertoire.
Article in Blood advances, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 43 citations in OpenAlex.
- Precision targeting of autoreactive B cells in systemic lupus erythematosus using anti-9G4 idiotope synthetic immune receptor T cells.bioRxiv : the preprint server for biology · 2025Article
- Clinicopathological, Genomic, and Transcriptomic Feature Analysis of Primary Adrenal Large B-cell Lymphoma: Insights Into Immune-privileged Sites.The American journal of surgical pathology · 2025Article
- Reliable detection of CNS lymphoma-derived circulating tumor DNA in cerebrospinal fluid using multi-biomarker NGS profiling: insights from a real-world study.Biomarker research · 2025Article
- Tirabrutinib for CNS relapsed primary vitreoretinal lymphoma.Annals of hematology · 2025Article
- Biological Findings and Clinical Outcomes in Patients Treated With R-CHOP Plus High-Dose Methotrexate as First-Line Therapy in Large B-Cell Lymphoma With Testis Involvement.European journal of haematology · 2025Article
- Intraocular Lymphoma: A Review.Journal of ophthalmic & vision research · 2025Review
- Distinct groups of autoantigens as drivers of ocular adnexal MALT lymphoma pathogenesis.Life science alliance · 2024Article
- A comprehensive overview of diagnosis, imaging and treatment of vitreoretinal lymphoma.European journal of ophthalmology · 2024Review
- Hyper-N-glycosylated SEL1L3 as auto-antigenic B-cell receptor target of primary vitreoretinal lymphomas.Scientific reports · 2024Article
- Untangling hairy cell leukaemia (HCL) variant and other HCL-like disorders: Diagnosis and treatment.Journal of cellular and molecular medicine · 2024Review
- Next-Generation Sequencing of Vitreoretinal Lymphoma by Vitreous Liquid Biopsy: Diagnostic Potential and Genotype/Phenotype Correlation.Investigative ophthalmology & visual science · 2023Article
- The 5th Edition of the World Health Organization Classification of Tumours of the Eye and Orbit.Ocular oncology and pathology · 2023Article
- Review
- Article
- Novel skewed usage of B-cell receptors in COVID-19 patients with various clinical presentations.Immunology letters · 2022Article
- Characterization of the Intra-tumoral B Cell Immunoglobulin Repertoire Is of Prognostic Value for Esophageal Squamous Cell Carcinoma.Frontiers in immunology · 2022Article
- Treatment Regimens for Immunocompetent Elderly Patients with Primary Central Nervous System Lymphoma: A Scoping Review.Cancers · 2021Article
- Vitreoretinal Lymphoma.Cancers · 2021Review
- Article
- Cytologic and Molecular Diagnostics for Vitreoretinal Lymphoma: Current Approaches and Emerging Single-Cell Analyses.Frontiers in molecular biosciences · 2020Review
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Authors and funding
19 authors at 6 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary vitreoretinal lymphoma (PVRL) is a high-grade lymphoma affecting the vitreous and/or the retina. The vast majority of cases are histopathologically classified as diffuse large B-cell lymphoma (DLBCL) and considered a subtype of primary central nervous system lymphoma (PCNSL). To obtain more insight into the ontogenetic relationship between PVRL and PCNSL, we adopted an immunogenetic perspective and explored the respective immunoglobulin gene repertoire profiles from 55 PVRL cases and 48 PCNSL cases. In addition, considering that both entities are predominantly related to activated B-cell (ABC) DLBCL, we compared their repertoire with that of publicly available 262 immunoglobulin heavy variable domain gene rearrangement sequences from systemic ABC-type DLBCLs. PVRL displayed a strikingly biased repertoire, with the IGHV4-34 gene being used in 63.6% of cases, which was significantly higher than in PCNSL (34.7%) or in DLBCL (30.2%). Further repertoire bias was evident by (1) restricted associations of IGHV4-34 expressing heavy chains, with κ light chains utilizing the IGKV3-20/IGKJ1 gene pair, including 5 cases with quasi-identical sequences, and (2) the presence of a subset of stereotyped IGHV3-7 rearrangements. All PVRL IGHV sequences were highly mutated, with evidence of antigen selection and ongoing mutations. Finally, half of PVRL and PCNSL cases carried the MYD88 L265P mutation, which was present in all 4 PVRL cases with stereotyped IGHV3-7 rearrangements. In conclusion, the massive bias in the immunoglobulin gene repertoire of PVRL delineates it from PCNSL and points to antigen selection as a major driving force in their development.
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