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ORPHA:86884

Subcutaneous panniculitis-like T-cell lymphoma

low confidenceDisorder

Also known as: SPTCL · Subcutaneous panniculitic T-cell lymphoma

Publications

1,165

Trials

9

Interventional, condition-specific

Researchers

1,166

Distinct authors in sample

Gene link

HAVCR2

Definitive

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare cytotoxic cutaneous alpha-beta T-cell lymphoma characterized by solitary or multiple erythematous subcutaneous nodules and plaques that can be localized to the lower extremities or generalized. It has been recognized as a distinct subset of peripheral T-cell lymphomas originating and presenting primarily in the subcutaneous fat tissue and often associated with hemophagocytic lymphohistiocytosis.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (4)

subcutaneous panniculitic T-cell lymphoma · subcutaneous panniculitis-like T-cell lymphoma · subcutaneous panniculitis-like T-cell lymphoma (Alpha/Beta type) · subcutaneous panniculitis-like T-cell lymphoma, Alpha/Beta type

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 identifiedPresent

    Definitive — HAVCR2

  2. LiteraturePresent

    1,165 matched papers (698 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialPresent

    9 matched on ClinicalTrials.gov (2 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?

Yes — we know a specific gene responsible (HAVCR2).

GenCC classification: Definitive.

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?

1,165

1,165 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.

1,165 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).

698 in the last 10 years · low confidence

Phrase hits: 1,165 · MeSH hits: 18

Open Europe PMC search

Who's working on it?

1,166

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
    Zhang Y7 papers · 2026

    Medical Oncology Department, Pediatric Oncology Center, Beijing Children's Hospital, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education, National Key Discipline of Pediatrics, Capital Medical University, Beijing, 100045, People's Republic of China.

    Papers in Europe PMC
  2. 02
    Polprasert C5 papers · 2024

    Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Thailand; Center of Excellence in Translational Hematology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand. Electronic address: chantana.po@chula.ac.th.

    Papers in Europe PMC
  3. 03
    Wang T5 papers · 2026

    Medical Oncology Department, Pediatric Oncology Center, Beijing Children's Hospital, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education, National Key Discipline of Pediatrics, Capital Medical University, Beijing, 100045, People's Republic of China. wangtianyou@bch.com.cn.

    Papers in Europe PMC
  4. 04
    Chen C4 papers · 2025

    Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

    Papers in Europe PMC
  5. 05
    Chen L4 papers · 2026

    Department of Pathology, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China.

    Papers in Europe PMC
  6. 06
    Wang W4 papers · 2025

    Department of Dermatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, People's Republic of China.

    Papers in Europe PMC
  7. 07
    Wang Y4 papers · 2026

    Ultrasound Medicine Center, Gansu Provincial Maternity and Child-Care Hospital, Lanzhou, China.

    Papers in Europe PMC
  8. 08
    Zhang H4 papers · 2026

    Department of Hematology/Oncology, Guangzhou Women and Children's Medical Center, Guangzhou, China.

    Papers in Europe PMC
  9. 09
    Zhang L4 papers · 2026

    Dermatology Hospital, Southern Medical University, Guangzhou, Guangdong Province, China. zhanglian1@smu.edu.cn.

    Papers in Europe PMC
  10. 10
    Zhang R4 papers · 2025

    State Key Laboratory of Experimental Hematology, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, National Clinical Research Center for Blood Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

9

interventional trials for this specific condition

9 interventional trials matched this specific condition name; 2 currently recruiting in our sample.

Data as of 27 July 2026

9 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 91.2th percentile).

low confidence · 91.2th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

9 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

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 2026-07-26

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.

"Subcutaneous panniculitis-like T-cell lymphoma" OR "SPTCL" OR "Subcutaneous panniculitic T-cell lymphoma" OR "subcutaneous panniculitis-like T-cell lymphoma (Alpha/Beta type)" OR "subcutaneous panniculitis-like T-cell lymphoma, Alpha/Beta type"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Subcutaneous panniculitis-like T-cell lymphoma

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

"Subcutaneous panniculitis-like T-cell lymphoma" OR "SPTCL" OR "Subcutaneous panniculitic T-cell lymphoma" OR "subcutaneous panniculitis-like T-cell lymphoma (Alpha/Beta type)" OR "subcutaneous panniculitis-like T-cell lymphoma, Alpha/Beta type" OR "HAVCR2"

Recall-expansion terms: HAVCR2

Interventional trials matched via: both (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 9 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase, mesh, recall-expansion; with hits: phrase, mesh

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 (1165) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T03:16:48.806Z