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 found
No HPO disease–phenotype associations via Monarch for these Mondo IDs
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- 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)
None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”
Animal models (Monarch / Alliance)
None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.
Monarch fetch 2026-07-29
Therapies
Designations, candidates, and chemicals
FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.
Orphan designation (FDA · EMA)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
No drugs or clinical candidates returned for this Mondo ID on Open Targets.
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
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=3967) is 59.
1,620 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).
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 11 September 2026
65 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 97.8th percentile).
low confidence · 97.8th 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
Not reviewed·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.
Other registries (secondary)
Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.
raw 1 · after dedupe 1 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 1 · dropped 0 · fetched 2026-07-30
Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri
No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).
Uncertain / not reviewed (1)
Where to find support
Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.
Orphanet entry for Primary vitreoretinal large B-cell lymphoma — check Associations / patient organisations on that page.
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