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 checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- 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)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
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=5449) is 41.
1,378 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
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 27 July 2026
67 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 97.8th percentile).
low confidence · 97.8th 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
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
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)
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
Conditions: T-cell Lymphoma · NK-Cell Lymphoma · T-cell Prolymphocytic Leukemia · T-cell Large Granular Lymphocytic Leukemia·Matched via name phrase
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
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