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ORPHA:289661

Epstein-Barr virus-positive diffuse large B-cell lymphoma

high confidenceDisorder

Also known as: EBV-positive DLBCL · Epstein-Barr virus-positive diffuse large B-cell lymphoma not otherwise specified · Epstein-Barr virus-positive diffuse large B-cell lymphoma, NOS

Publications

849

85.4th percentile

Trials

13

Interventional, condition-specific

Researchers

1,352

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly is a rare form of diffuse large B-cell lymphoma occurring most commonly in patients over the age of 50 (usually between 70-75 years of age), without overt immunodeficiency, and presenting with nodal and extranodal involvement (in sites such as the stomach, lung, skin and pancreas) and B symptoms (fever, night sweats, weight loss). The tumor is characterized by an aggressive course and a short survival rate.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (6)

EBV Positive diffuse large B-cell lymphoma of the elderly · EBV-Positive diffuse large B-cell lymphoma, NOS · EBV-positive DLBCL of the elderly · Epstein-Barr Virus Positive diffuse large B-cell lymphoma of the elderly · Senile EBV-associated B-cell lymphoproliferative disorder · age-related EBV Positive B-cell lymphoproliferative disorder

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.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    849 matched papers (654 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    13 matched on ClinicalTrials.gov (3 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?

849

849 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.

849 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).

654 in the last 10 years · high confidence · 85.4th percentile (publications denominator)

Phrase hits: 849 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,352

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.

  1. 01
    Wang L7 papers · 2026

    Department of Pathology, The First People's Hospital of Foshan, Foshan, 528000, People's Republic of China.

    Papers in Europe PMC
  2. 02
    Beltran BE5 papers · 2018

    Department of Oncology and Radiotherapy, Edgardo Rebagliati Martins Hospital, Lima, Peru.

    Papers in Europe PMC
  3. 03
    Castillo JJ5 papers · 2018

    Division of Hematologic Malignancies, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.

    Papers in Europe PMC
  4. 04
    Kim WS5 papers · 2026

    Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81, Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea. wskimsmc@skku.edu.

    Papers in Europe PMC
  5. 05
    Kim SJ4 papers · 2023

    Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81, Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.

    Papers in Europe PMC
  6. 06
    Liu X4 papers · 2022

    Department of Pathology, The First Affliated Hospital, Zhejiang University Medical College Hangzhou, China.

    Papers in Europe PMC
  7. 07
    Miranda RN4 papers · 2016

    Department of Hematopathology, the University of Texas MD Anderson Cancer Center, Houston, Texas.

    Papers in Europe PMC
  8. 08
    Pileri SA4 papers · 2022

    Haematopathology Division, European Institute of Oncology-IEO IRCCS Milan, 20141 Milan, Italy.

    Papers in Europe PMC
  9. 09
    Quintanilla-Martinez L4 papers · 2026

    b Department of Pathology , University of Tübingen , Germany.

    Papers in Europe PMC
  10. 10
    Sotomayor EM4 papers · 2018

    Division of Hematology/Oncology, George Washington University Cancer Center, Washington, DC, USA.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

13

interventional trials for this specific condition

13 interventional trials matched this specific condition name; 3 currently recruiting in our sample. 1,014 trials are registered for diffuse large B-cell lymphoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

13 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 93.5th percentile).

high confidence · 93.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

13 interventional trials matched after quoted-phrase search and title/condition post-filter.

Broader category: diffuse large B-cell lymphoma

1,014

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

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 3 · after dedupe 3 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 3 · 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 (3)

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 Epstein-Barr virus-positive diffuse large B-cell lymphoma — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This 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.

"Epstein-Barr virus-positive diffuse large B-cell lymphoma" OR "EBV-positive DLBCL" OR "Epstein-Barr virus-positive diffuse large B-cell lymphoma not otherwise specified" OR "Epstein-Barr virus-positive diffuse large B-cell lymphoma, NOS" OR "EBV Positive diffuse large B-cell lymphoma of the elderly" OR "EBV Positive diffuse large B-cell lymphoma of elderly" OR "EBV-Positive diffuse large B-cell lymphoma, NOS" OR "EBV-positive DLBCL of the elderly" OR "EBV-positive DLBCL of elderly" OR "Epstein-Barr Virus Positive diffuse large B-cell lymphoma of the elderly" OR "Epstein-Barr Virus Positive diffuse large B-cell lymphoma of elderly" OR "Senile EBV-associated B-cell lymphoproliferative disorder" OR "age-related EBV Positive B-cell lymphoproliferative disorder"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Epstein-Barr virus-positive diffuse large B-cell lymphoma" OR "EBV-positive DLBCL" OR "Epstein-Barr virus-positive diffuse large B-cell lymphoma not otherwise specified" OR "Epstein-Barr virus-positive diffuse large B-cell lymphoma, NOS" OR "EBV Positive diffuse large B-cell lymphoma of the elderly" OR "EBV Positive diffuse large B-cell lymphoma of elderly" OR "EBV-Positive diffuse large B-cell lymphoma, NOS" OR "EBV-positive DLBCL of the elderly" OR "EBV-positive DLBCL of elderly" OR "Epstein-Barr Virus Positive diffuse large B-cell lymphoma of the elderly" OR "Epstein-Barr Virus Positive diffuse large B-cell lymphoma of elderly" OR "Senile EBV-associated B-cell lymphoproliferative disorder" OR "age-related EBV Positive B-cell lymphoproliferative disorder"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 13 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"diffuse large B-cell lymphoma"

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T12:16:39.947Z