ORPHA:695631
Primary vitreoretinal large B-cell lymphoma
Also known as: PVLR · PVR-LBCL · Primary diffuse large B-cell lymphoma of the virtreoretina
Publications
1,620
Trials
65
Interventional, condition-specific
Researchers
1,012
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0004351
- MeSH:D064090
- UMLS:C0281658
- NCIT:C9184
Additional Mondo synonyms (4)
PIOL · intraocular lymphoma · primary intraocular lymphoma · primary intraocular non-Hodgkin's lymphoma
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
2/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
1,620 matched papers (926 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialPresent
65 matched on ClinicalTrials.gov (1 recruiting in sample)
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
1,620
1,620 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
1,620 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
926 in the last 10 years · low confidence
Phrase hits: 1,620 · MeSH hits: 35
Who's working on it?
1,012
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Tanaka T6 papers · 2026
Department of Ophthalmology, Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, Brazil.
Papers in Europe PMC - 02Heindl LM5 papers · 2024
Department of Ophthalmology, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany.
Papers in Europe PMC - 03Matsuo T5 papers · 2025
Regenerative and Reconstructive Medicine (Ophthalmology), Okayama University Graduate School of Interdisciplinary Science and Engineering in Health Systems, Okayama City, Japan.
Papers in Europe PMC - 04Hosseini SM4 papers · 2022
Eye Research Center, Mashhad University of Medical Sciences, Mashhad, Iran. smaryam_hosseini@yahoo.com.
Papers in Europe PMC - 05Kakkassery V4 papers · 2026
Klinik für Augenheilkunde, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland.
Papers in Europe PMC - 06Wang L4 papers · 2026
Department of Hematology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Papers in Europe PMC - 07Wang X4 papers · 2026
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
Papers in Europe PMC - 08Zamani G4 papers · 2022
Eye Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Papers in Europe PMC - 09Zhang X4 papers · 2026
Department of Ophthalmology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
Papers in Europe PMC - 10Fend F3 papers · 2026
Institut für Pathologie und Neuropathologie, Universitätsklinikum Tübingen, Liebermeisterstr. 8, 72076, Tübingen, Deutschland. Falko.fend@med.uni-tuebingen.de.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
65
interventional trials for this specific condition
65 interventional trials matched this specific condition name; 1 currently recruiting in our sample.
Data as of 27 July 2026
65 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 97.7th percentile).
low confidence · 97.7th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
65 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT06973811·NOT YET RECRUITING·ZR-MTX for PIOL Phase II Trial
Conditions: Primary Intraocular Lymphoma·Matched via name + MeSH
Observational and natural-history studies
2 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.
None of the matched observational studies is currently listed as recruiting.
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Primary vitreoretinal large B-cell lymphoma" OR "PVR-LBCL" OR "Primary diffuse large B-cell lymphoma of the virtreoretina" OR "Primary diffuse large B-cell lymphoma of virtreoretina" OR "intraocular lymphoma" OR "primary intraocular lymphoma" OR "primary intraocular non-Hodgkin's lymphoma"
MeSH descriptor terms unioned into the query: Intraocular Lymphoma
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Primary vitreoretinal large B-cell lymphoma" OR "PVR-LBCL" OR "Primary diffuse large B-cell lymphoma of the virtreoretina" OR "Primary diffuse large B-cell lymphoma of virtreoretina" OR "intraocular lymphoma" OR "primary intraocular lymphoma" OR "primary intraocular non-Hodgkin's lymphoma"
Interventional trials matched via: both (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 65 interventional · 2 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: PVLR; PIOL
Confidence reasoning
- Preferred label is multi-word and distinctive
- 2 synonym(s) dropped by stoplist (may under-count)
- No label/synonym collisions with other diseases in this corpus
- Publication count (1620) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity
Ingested 2026-07-27T20:42:33.271Z