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subcutaneous panniculitis-like T-cell lymphoma (SPTCL) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

subcutaneous panniculitis-like T-cell lymphoma

MONDO:0019475

Also known as:SPTCLsubcutaneous panniculitic T-cell lymphomasubcutaneous panniculitis-like T-cell lymphoma (Alpha/Beta type)

Find Clinical TrialsTrials

Overview

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic cutaneous lymphoma that has been recognized as a distinct subset of peripheral T-cell lymphomas originating and presenting primarily in the subcutaneous fat tissue.

GeneticsAutosomal recessive · HAVCR2 (chr 5)

Clinical Trials5 registered · 2 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include always present findings: Subcutaneous panniculitis-like T-cell lymphoma and Panniculitis; and rarely findings: Autoimmunity, Facial edema, Hemophagocytosis, and Low red blood cell count (anemia) and others. 12 total HPO annotations.

Organ System	Phenotype Count	Example Features
Blood and immune system	5	Autoimmunity, Subcutaneous panniculitis-like T-cell lymphoma, Low red blood cell count (anemia)

Genetic Landscape

Assembled from HGNC gene records, ClinGen gene-disease validity evidence, and ClinVar pathogenic variant data. Validity classifications reflect the strength of published evidence linking each gene to this disease.

HAVCR2 encodes hepatitis A virus cellular receptor 2 (301 aa). Cell surface receptor implicated in modulating innate and adaptive immune responses. Generally accepted to have an inhibiting function. Highest expression in Lung (25.9 TPM) and Spleen (24.7 TPM).

Subcutaneous panniculitis-like T-cell lymphoma is associated with mutations in the HAVCR2 gene on chromosome 5.

HAVCR2 is classified as a druggable target (Cell Surface, Clinically Actionable, and Drug Resistance categories) with score 17.4.

Diagnosis

Genetic testing for HAVCR2 is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for subcutaneous panniculitis-like T-cell lymphoma has been reported in the published literature.

Prognosis

Phenotype severity distribution: 2 always present features.

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

5 clinical trials registered, 2 recruiting. Interventions under study include drug therapy, other interventions, procedural interventions, and biologic therapy. Pipeline includes 3 PHASE1, 1 NA. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT05978141](https://clinicaltrials.gov/study/NCT05978141)

Data assembled from 7 of 12 sources · Last updated Sep 18, 2026, 2:36 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:618398

Orphanet

European rare disease database

Orphanet:86884

GARD

Genetic and Rare Diseases Info Center

GARD:10193

Questions for Your Care Team

→Should my partner and I meet with a genetic counselor to discuss carrier status and family planning?
→Are any of the 5 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Patient case studies	36	47%
Disease patterns and progression	10	13%
Research summaries	9	12%
Laboratory research	8	11%
Clinical study results	6	8%
New treatment approaches	3	4%
Other research	2	3%
Testing and diagnosis research	2	3%

Kisho

subcutaneous panniculitis-like T-cell lymphoma

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

Latest News & Developments

Prognostic Value of Skin Lesion Characteristics in Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Retrospective Cohort Study Assessing the Applicability of T Category of the TNM Classification for Primary Cutaneous Lymphomas Other Than Mycosis Fungoides and Sézary Syndrome.

HAVCR2 in subcutaneous panniculitis like T cell lymphoma.

subcutaneous panniculitis-like T-cell lymphoma

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

Latest News & Developments

Prognostic Value of Skin Lesion Characteristics in Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Retrospective Cohort Study Assessing the Applicability of T Category of the TNM Classification for Primary Cutaneous Lymphomas Other Than Mycosis Fungoides and Sézary Syndrome.

HAVCR2 in subcutaneous panniculitis like T cell lymphoma.