ORPHA:86882
Hepatosplenic T-cell lymphoma
Publications
1,378
Trials
67
Interventional, condition-specific
Researchers
1,400
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
A rare T-cell non-Hodgkin lymphoma characterized by a proliferation of cytotoxic T-cells, usually gamma delta T-cells, with involvement of the liver and spleen, but without involvement of lymph nodes. The bone marrow is consistently affected. Patients typically present during adolescence or young adulthood with , pancytopenia, and systemic symptoms. Peripheral blood involvement may develop later in the disease course. There is a clear male preponderance. The disease often occurs in the context of long-term immunosuppression. The course is aggressive with poor therapy response.
How rare: How common this is has not been clearly measured.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0019474
- UMLS:C1333984
- NCIT:C8459
Additional Mondo synonyms (1)
Hepatosplenic gamma/Delta 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.”
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
1,378 matched papers (796 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
67 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
13
Drugs / clinical candidates · MONDO_0019474
- CISPLATIN·phase 2
- CYCLOPHOSPHAMIDE·phase 2
- DOXORUBICIN·phase 2
- GEMCITABINE·phase 2
- LENALIDOMIDE·phase 2
- METHYLPREDNISOLONE·phase 2
- PREDNISOLONE·phase 2
- VINCRISTINE·phase 2
- FLUDARABINE·phase 1
- DOXORUBICIN HYDROCHLORIDE·phase 1 2
- ETOPOSIDE·phase 1 2
- PREDNISONE·phase 1 2
- VINCRISTINE SULFATE·phase 1 2
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
Literature
Is anyone studying this?
1,378
1,378 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.
1,378 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).
796 in the last 10 years · low confidence
Phrase hits: 1,378 · MeSH hits: 0
Who's working on it?
1,400
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.
- 01Kim WS6 papers · 2025
Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Papers in Europe PMC - 02Zhang H6 papers · 2026
National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, and Department of Lymphoma, Tianjin Medical University Cancer Institute & Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin 300060, China.
Papers in Europe PMC - 03de Leval L5 papers · 2025
Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, 25 rue du Bugnon, CH- 1011, Lausanne, Switzerland. Laurence.deLeval@chuv.ch.
Papers in Europe PMC - 04Dogan A5 papers · 2025
Hematopathology Service, Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
Papers in Europe PMC - 05Gaulard P5 papers · 2026
Institut Mondor de Recherche Biomédicale, INSERM U955, Université Paris Est Créteil, Créteil, France.
Papers in Europe PMC - 06Horwitz SM5 papers · 2025
Department of Medicine, Lymphoma Service, Memorial Sloan Kettering Cancer Center, New York, NY.
Papers in Europe PMC - 07Liu Y5 papers · 2025
Shanghai Institute of Hematology, State Key Laboratory of Medical Genomics, National Research Center for Translational Medicine at Shanghai, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Papers in Europe PMC - 08Wang J5 papers · 2025
Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Papers in Europe PMC - 09
- 10
Clinical research
Is a treatment being tested?
67
interventional trials for this specific condition
67 interventional trials matched this specific condition name; 3 currently recruiting in our sample.
Data as of 11 September 2026
67 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 97.9th percentile).
low confidence · 97.9th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
67 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT07691450·NOT YET RECRUITING·Belinostat in Combination With Azacitidine or Pralatrexate for the Treatment of Relapse or Refractory T-cell Lymphoma
Not reviewed·Conditions: Recurrent Anaplastic Large Cell Lymphoma · Recurrent Enteropathy-Associated T-Cell Lymphoma · Recurrent Follicular Helper T-Cell Lymphoma · Recurrent Follicular Helper T-Cell Lymphoma, Angioimmunoblastic-Type·Matched via name phrase
- NCT05475925·RECRUITING·A Study of DR-01 in Subjects With Large Granular Lymphocytic Leukemia or Cytotoxic Lymphomas
Not reviewed·Conditions: LGLL - Large Granular Lymphocytic Leukemia · Primary Cutaneous Gamma-Delta T-Cell Lymphoma · Primary Cutaneous CD8+ Aggressive Epidermotropic T-Cell Lymphoma · Hepatosplenic T-cell Lymphoma·Matched via name phrase
- NCT07055477·RECRUITING·A Phase I Trial Anti-CC Chemokine Receptor 4 Chimeric Antigen Receptor T Cells (CCR4 CAR T Cells) for CCR4 Expressing T-cell Malignancies Including Peripheral T-cell Non-Hodgkin Lymphoma (PTCL) and Cutaneous T-cell Non-Hodgkin Lymphoma (CTCL)
Not reviewed·Conditions: Relapsed and/or Refractory Mature T Cell Malignancy · Peripheral T-Cell Lymphoma · Angioimmunoblastic T-cell Lymphoma · Anaplastic Large Cell Lymphoma·Matched via name phrase
Observational and natural-history studies
3 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
- NCT05978141·RECRUITING·A Registry for People With T-cell Lymphoma
Not reviewed·Conditions: T-cell Lymphoma · NK-Cell Lymphoma · T-cell Prolymphocytic Leukemia · T-cell Large Granular Lymphocytic Leukemia·Matched via name phrase
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 2 · after dedupe 2 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 2 · 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 (2)
- isrctn·ISRCTN42054893·No longer recruiting·A study to determine the maximum tolerated dose and activity of the combination of romidepsin and carfilzomib in relapsed or refractory peripheral T-cell lymphoma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN52265296·No longer recruiting·Alemtuzumab, MabCampath® with 2-weekly CHOP chemotherapy for mature T-cell non-Hodgkin's lymphoma
skipped — LLM skipped (--skip-llm)
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 Hepatosplenic T-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.
"Hepatosplenic T-cell lymphoma" OR "Hepatosplenic gamma/Delta T-cell lymphoma"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Hepatosplenic T-cell lymphoma" OR "Hepatosplenic gamma/Delta T-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: 67 interventional · 3 observational · 0 expanded access. Only interventional studies enter the trial headline.
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
- Publication count (1378) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity
Ingested 2026-07-27T03:16:36.390Z