RARE DISEASERESEARCH ATLAS
Open navigation menu

ORPHA:86880

Enteropathy-associated T-cell lymphoma

low confidenceDisorder

Also known as: EATL · ETTL · Enteropathy-associated T-cell lymphoma type 1 · Enteropathy-type T-cell lymphoma · Intestinal T-cell lymphoma

Publications

2,198

Trials

28

Interventional, condition-specific

Researchers

1,305

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare T-cell non-Hodgkin lymphoma characterized by a neoplasm of intraepithelial T-cells mostly occurring in the jejunum or ileum in patients with celiac disease. The lesion may be multifocal and form ulcerating nodules, plaques, strictures, or an exophytic mass. The mesentery and mesenteric lymph nodes are commonly involved. Patients typically present with abdominal pain, malabsorption or diarrhea, anorexia, weight loss, fatigue, nausea, vomiting, and sometimes intestinal perforation or hemorrhage. Prognosis is generally poor.

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.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (5)

EATL, type I · enteropathy associated T-cell lymphoma · enteropathy-associated T-cell lymphoma, type I · enteropathy-type T-cell lymphoma · intestinal T-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

    2,198 matched papers (1,249 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 designationPresent

    2 FDA designations (1 FDA orphan-indication approval) — e.g. brentuximab vedotin Source

  6. Interventional trialPresent

    28 matched on ClinicalTrials.gov (4 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)

2

Designations · 1 with FDA orphan-indication approval

  • FDA brentuximab vedotinEnteropathy-Associated T-Cell Lymphoma · 2016-07-07 · Not FDA Approved for Orphan Indication
  • FDA brentuximab vedotin (Adcetris)Angioimmunoblastic T-cell lymphoma adult T-cell leukemia/lymphoma Peripheral T-Cell Lymphoma Enteropathy-Associated T-Cell Lymphoma Anaplastic Large Cell Lymphoma · 2008-10-23

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

26

Drugs / clinical candidates · MONDO_0019473

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

2,198

2,198 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.

2,198 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).

1,249 in the last 10 years · low confidence

Phrase hits: 2,198 · MeSH hits: 36

Open Europe PMC search

Who's working on it?

1,305

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
    Gaulard P10 papers · 2026

    AP-HP, Henri Mondor Hospital, Pathology Department, F-94010 Créteil, France; University Paris Est Créteil, INSERM, IMRB, F-94010 Créteil.

    Papers in Europe PMC
  2. 02
    de Leval L8 papers · 2026

    Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Lausanne. Laurence.deleval@chuv.ch.

    Papers in Europe PMC
  3. 03
    Chambers JK7 papers · 2026

    Laboratory of Veterinary Pathology, Department of Veterinary Medical Sciences, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Bunkyo-ku, Tokyo, Japan.

    Papers in Europe PMC
  4. 04
    Uchida K7 papers · 2026

    Laboratory of Veterinary Pathology, Department of Veterinary Medical Sciences, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Bunkyo-ku, Tokyo, Japan.

    Papers in Europe PMC
  5. 05
    Lemonnier F6 papers · 2026

    University Paris Est Créteil, INSERM, IMRB, F-94010 Créteil, France; AP-HP, Henri Mondor Hospital, Lymphoid malignancies unit, F-94010 Créteil.

    Papers in Europe PMC
  6. 06
    Zhang Y6 papers · 2026

    Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, P.R. China.

    Papers in Europe PMC
  7. 07
    Drieux F5 papers · 2026

    Centre Henri Becquerel, Service of Anatomical and Cytological Pathology, Centre Henri Becquerel Rouen, France, Rouen.

    Papers in Europe PMC
  8. 08
    Goto-Koshino Y5 papers · 2026

    Veterinary Medical Center, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Bunkyo-ku, Tokyo, Japan.

    Papers in Europe PMC
  9. 09
    Nakashima K5 papers · 2026

    Japan Small Animal Medical Center, Tokorozawa, Saitama, Japan.

    Papers in Europe PMC
  10. 10
    Poullot E5 papers · 2026

    AP-HP, Henri Mondor Hospital, Pathology Department, F-94010 Créteil.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

28

interventional trials for this specific condition

28 interventional trials matched this specific condition name; 4 currently recruiting in our sample.

Data as of 11 September 2026

28 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 95.8th percentile).

low confidence · 95.8th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

1 observational study matches 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 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 Enteropathy-associated T-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.

"Enteropathy-associated T-cell lymphoma" OR "Enteropathy-associated T-cell lymphoma type 1" OR "Enteropathy-type T-cell lymphoma" OR "Intestinal T-cell lymphoma" OR "EATL, type I" OR "enteropathy associated T-cell lymphoma" OR "enteropathy-associated T-cell lymphoma, type I"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Enteropathy-Associated T-Cell Lymphoma

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

"Enteropathy-associated T-cell lymphoma" OR "Enteropathy-associated T-cell lymphoma type 1" OR "Enteropathy-type T-cell lymphoma" OR "Intestinal T-cell lymphoma" OR "EATL, type I" OR "enteropathy associated T-cell lymphoma" OR "enteropathy-associated T-cell lymphoma, type I"

Interventional trials matched via: both, 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: 28 interventional · 1 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: EATL; ETTL

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 (2198) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity

Ingested 2026-07-27T03:16:27.009Z