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ORPHA:86884

Subcutaneous panniculitis-like T-cell lymphoma

low confidenceDisorder

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

Publications

10,870

Trials

9

Interventional, condition-specific

Researchers

1,166

Distinct authors in sample

Gene link

HAVCR2

Definitive

Readiness

4/6

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.”

4/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedPresent

    Definitive — HAVCR2

  2. LiteraturePresent

    10,870 matched papers (9,708 in last 10 years) Source

  3. Phenotype characterisedPresent

    23 HPO annotations (e.g. Hepatosplenomegaly; Weight loss; Fever) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  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)

23

Associated phenotypes · MONDO:0019475

  • Hepatosplenomegaly
  • Weight loss
  • Fever
  • Abnormality of the coagulation cascade
  • Erythematous plaque

Showing 5 of 23 — open Monarch for the full list.

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

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

10,870

10,870 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.

10,870 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).

9,708 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 11 September 2026 · last trial check 28 July 2026

9 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 92th percentile).

low confidence · 92th 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

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)

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 Subcutaneous panniculitis-like 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.

("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 (MESH:"Subcutaneous panniculitis-like T-cell lymphoma") OR ("HAVCR2" OR "HAVCR2 syndrome" OR "HAVCR2-related")

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"

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; 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 (10870) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

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