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ORPHA:98838

Primary mediastinal large B-cell lymphoma

medium confidenceDisorder

Also known as: Large cell lymphoma of the mediastinum · Med-DLBCL · Mediastinal diffuse large-cell lymphoma with sclerosis · Primary mediastinal clear cell lymphoma of B-cell type

Publications

2,342

90.1th percentile

Trials

100

Interventional, condition-specific

Researchers

1,521

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare subtype of diffuse large B-cell lymphoma (DLBCL), arising from B cells of thymic origin, predominantly affecting women between the ages of 20-30, and that usually presents with a bulky and rapidly expanding anterior mediastinal mass, often with pleural and pericardial effusions, and that can invade the lungs, superior vena cava, pleura, pericardium, and chest wall, leading to manifestations of cough, dyspnea, and superior vena cava syndrome.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (12)

B-cell diffuse large cell lymphoma of mediastinum · B-cell diffuse large cell lymphoma of the mediastinum · PMBL · PMLCL · large cell lymphoma of the mediastinum · mediastinal (thymic) large B-cell lymphoma · mediastinal B-cell diffuse large cell lymphoma · mediastinal diffuse large cell lymphoma with sclerosis · mediastinal diffuse large-cell lymphoma with sclerosis · mediastinal large B-cell lymphoma · primary mediastinal clear cell lymphoma of B-cell type · primary mediastinal large B-cell 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.”

3/6 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

    2,342 matched papers (1,778 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 designationPartial

    1 FDA · 2 EMA designations (none yet with FDA orphan-indication approval) — e.g. autologous T cells transduced with retroviral vector encoding an anti-CD19 CD28/CD3-zeta chimeric antigen receptor (axicabtagene ciloleucel) Source

  6. Interventional trialPresent

    100 matched on ClinicalTrials.gov (35 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)

3

Designations · no FDA orphan-indication approval yet

  • EMA autologous T cells transduced with retroviral vector encoding an anti-CD19 CD28/CD3-zeta chimeric antigen receptor (axicabtagene ciloleucel) (Yescarta)Treatment of primary mediastinal large B-cell lymphoma · 09/10/2015 · PositiveEMA designation
  • FDA lisocabtagene maraleucel (Breyanzi)Primary Mediastinal Large B-Cell Lymphoma · 2018-07-12
  • EMA lisocabtagene maraleucelTreatment of primary mediastinal large B-cell lymphoma · 19/11/2018 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

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?

2,342

2,342 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.

2,342 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).

1,778 in the last 10 years · medium confidence · 90.1th percentile (publications denominator)

Phrase hits: 2,342 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,521

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
    Camus V6 papers · 2026

    Department of Hematology, Centre Henri Becquerel, Rouen, France.

    Papers in Europe PMC
  2. 02
    Vassilakopoulos TP6 papers · 2025

    Department of Haematology and Bone Marrow Transplantation, National and Kapodistrian University of Athens, School of Medicine, Laikon General Hospital, Athens 11527, Greece.

    Papers in Europe PMC
  3. 03
    Wang Y6 papers · 2026

    Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Department of Lymphoma, Peking University Cancer Hospital & Institute, Beijing 100142, China.

    Papers in Europe PMC
  4. 04
    Piperidou A5 papers · 2025

    Department of Haematology and Bone Marrow Transplantation, National and Kapodistrian University of Athens, School of Medicine, Laikon General Hospital, Athens 11527, Greece.

    Papers in Europe PMC
  5. 05
    Savage KJ5 papers · 2026

    BC Cancer, Vancouver, BC, Canada.

    Papers in Europe PMC
  6. 06
    Angelopoulou MK4 papers · 2025

    Department of Haematology and Bone Marrow Transplantation, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece.

    Papers in Europe PMC
  7. 07
    Bouzani M4 papers · 2025

    Department of Hematology and Lymphoma, Evangelismos General Hospital, Athens, Greece.

    Papers in Europe PMC
  8. 08
    Jardin F4 papers · 2026

    Department of Hematology, Centre Henri Becquerel, Rouen, France.

    Papers in Europe PMC
  9. 09
    Scott DW4 papers · 2026

    Centre for Lymphoid Cancer, BC Cancer, Vancouver, BC V5Z 1L3, Canada.

    Papers in Europe PMC
  10. 10
    Tsirigotis P4 papers · 2025

    Second Department of Internal Medicine, Propaedeutic, Hematology Unit, National and Kapodistrian University of Athens, University General Hospital "Attikon," Greece.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

100

interventional trials for this specific condition

100 interventional trials matched this specific condition name; 35 currently recruiting in our sample.

Data as of 11 September 2026

100 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 98.5th percentile).

medium confidence · 98.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

11 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.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

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 22 · after dedupe 22 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 22 · 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 (22)

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 mediastinal 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.

"Primary mediastinal large B-cell lymphoma" OR "Large cell lymphoma of the mediastinum" OR "Large cell lymphoma of mediastinum" OR "Med-DLBCL" OR "Mediastinal diffuse large-cell lymphoma with sclerosis" OR "Primary mediastinal clear cell lymphoma of B-cell type" OR "Primary mediastinal clear cell lymphoma of the B-cell type" OR "B-cell diffuse large cell lymphoma of mediastinum" OR "B-cell diffuse large cell lymphoma of the mediastinum" OR "PMLCL" OR "mediastinal (thymic) large B-cell lymphoma" OR "mediastinal B-cell diffuse large cell lymphoma" OR "mediastinal diffuse large cell lymphoma with sclerosis" OR "mediastinal large B-cell lymphoma"

Run this search on Europe PMC

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

"Primary mediastinal large B-cell lymphoma" OR "Large cell lymphoma of the mediastinum" OR "Large cell lymphoma of mediastinum" OR "Med-DLBCL" OR "Mediastinal diffuse large-cell lymphoma with sclerosis" OR "Primary mediastinal clear cell lymphoma of B-cell type" OR "Primary mediastinal clear cell lymphoma of the B-cell type" OR "B-cell diffuse large cell lymphoma of mediastinum" OR "B-cell diffuse large cell lymphoma of the mediastinum" OR "PMLCL" OR "mediastinal (thymic) large B-cell lymphoma" OR "mediastinal B-cell diffuse large cell lymphoma" OR "mediastinal diffuse large cell lymphoma with sclerosis" OR "mediastinal large B-cell lymphoma"

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: 100 interventional · 11 observational · 1 expanded access. Only interventional studies enter the trial headline.

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: PMBL

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T05:32:42.493Z