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ORPHA:86886

Angioimmunoblastic T-cell lymphoma

low confidenceDisorder

Also known as: Immunoblastic lymphadenopathy · Lymphogranulomatosis X · T-cell lymphoma, AILD type · AILT

Publications

18,524

Trials

123

Interventional, condition-specific

Researchers

1,243

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 infiltration of lymph nodes by neoplastic cells of T follicular helper cell origin with a polymorphous inflammatory background including markedly increased follicular dendritic cells and EBV-positive B-cells, as well as prominent proliferation of high endothelial venules. The spleen, liver, skin, and bone marrow are also frequently involved. Patients typically present with generalized lymphadenopathy, , systemic symptoms, and polyclonal hypergammaglobulinemia. Pruritic skin rash, arthritis, pleural effusion, and ascites may also be observed. The condition is aggressive with generally poor prognosis.

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 (7)

AILD · angioimmunoblastic T-cell lymphoma · angioimmunoblastic lymphadenopathy · angioimmunoblastic lymphadenopathy type T-cell lymphoma · angioimmunoblastic lymphadenopathy with Dysproteinemia · immunoblastic lymphadenopathy · lymphogranulomatosis X

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

    18,524 matched papers (9,571 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

    123 matched on ClinicalTrials.gov (7 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 vedotinAngioimmunoblastic T-cell lymphoma · 2013-09-13 · 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

42

Drugs / clinical candidates · MONDO_0004977

CTD chemicals (MyDisease.info)

2 associated chemicals · 108 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • BEAM regimen · therapeutic
  • Phenytoin · marker/mechanism

Pathways: Citrate cycle (TCA cycle); Cysteine and methionine metabolism; Glutathione metabolism; Metabolic pathways; Carbon metabolism; 2-Oxocarboxylic acid metabolism; Biosynthesis of amino acids; Ras signaling pathway

MyDisease.info · MONDO:0004977

Literature

Is anyone studying this?

18,524

18,524 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

18,524 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).

9,571 in the last 10 years · low confidence

Phrase hits: 18,524 · MeSH hits: 8

Open Europe PMC search

Who's working on it?

1,243

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 Y9 papers · 2026

    College of Veterinary Medicine, National Key Laboratory of Veterinary Public Health and Safety, China Agricultural University, Beijing, China.

    Papers in Europe PMC
  2. 02
    Li J7 papers · 2026

    Department of Hematology, Jiangsu Province Hospital, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China.

    Papers in Europe PMC
  3. 03
    Chen J6 papers · 2026

    Department of Nuclear Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, China. Electronic address: lindaxcx@vip.163.com.

    Papers in Europe PMC
  4. 04
    Li X6 papers · 2026

    Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Henan 450052 Zhengzhou, China; Lymphoma Diagnosis and Treatment Center of Henan Province, Zhengzhou, Henan 450052, China. Electronic address: lixiaoxin86@126.com.

    Papers in Europe PMC
  5. 05
    Wang J6 papers · 2026

    Department of Hematology, West China Hospital, Sichuan University, Chengdu, China.

    Papers in Europe PMC
  6. 06
    Wang X6 papers · 2026

    Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Henan 450052 Zhengzhou, China; Lymphoma Diagnosis and Treatment Center of Henan Province, Zhengzhou, Henan 450052, China.

    Papers in Europe PMC
  7. 07
    Wang H5 papers · 2026

    College of Veterinary Medicine, National Key Laboratory of Veterinary Public Health and Safety, China Agricultural University, Beijing, China.

    Papers in Europe PMC
  8. 08
    Wang L5 papers · 2026

    Department of Dermatovenereology, West China Hospital, Sichuan University, Chengdu, China.

    Papers in Europe PMC
  9. 09
    Wang Z5 papers · 2026

    Department of Gastroenterology, The Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, No. 1 Minde Rd., Nanchang 330006, Jiangxi, China.

    Papers in Europe PMC
  10. 10
    Li Y4 papers · 2026

    College of Veterinary Medicine, National Key Laboratory of Veterinary Public Health and Safety, China Agricultural University, Beijing, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

123

interventional trials for this specific condition

123 interventional trials matched this specific condition name; 7 currently recruiting in our sample.

Data as of 11 September 2026

123 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 98.7th percentile).

low confidence · 98.7th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

4 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 6 · after dedupe 6 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 6 · 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 (6)

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 Angioimmunoblastic 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.

"Angioimmunoblastic T-cell lymphoma" OR "Immunoblastic lymphadenopathy" OR "Lymphogranulomatosis X" OR "T-cell lymphoma, AILD type" OR "angioimmunoblastic lymphadenopathy" OR "angioimmunoblastic lymphadenopathy type T-cell lymphoma" OR "angioimmunoblastic lymphadenopathy with Dysproteinemia"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Immunoblastic Lymphadenopathy

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

"Angioimmunoblastic T-cell lymphoma" OR "Immunoblastic lymphadenopathy" OR "Lymphogranulomatosis X" OR "T-cell lymphoma, AILD type" OR "angioimmunoblastic lymphadenopathy" OR "angioimmunoblastic lymphadenopathy type T-cell lymphoma" OR "angioimmunoblastic lymphadenopathy with Dysproteinemia"

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: 123 interventional · 4 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: AILT; AILD

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 (18524) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity

Ingested 2026-07-27T03:17:14.926Z