subcutaneous panniculitis-like T-cell lymphoma
Findings
No curated finding names subcutaneous panniculitis-like T-cell lymphoma yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
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.
Definition from the Mondo Disease Ontology (MONDO:0019475), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal recessive inheritance
- Onset and course
- Late onset · Middle age onset · Juvenile onset · Young adult onset · Childhood onset
HPO, annotations 2026-09-02
Features
12 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- PanniculitisHPOHP:0012490
- 16 of 16 reported patients
- Very frequent (80% to 99% of cases)
- Subcutaneous panniculitis-like T-cell lymphomaHPOHP:0034403
- 16 of 16 reported patients
- Erythematous papuleHPOHP:0030350
- Very frequent (80% to 99% of cases)
- Abnormality of the coagulation cascadeHPOHP:0003256
- Frequent (30% to 79% of cases)
- ChillsHPOHP:0025143
- Frequent (30% to 79% of cases)
- Erythematous plaqueHPOHP:0025474
- Frequent (30% to 79% of cases)
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- HAVCR2HGNC:18437
- Definitive · G2P · Autosomal recessive · 2025
- Strong · PanelApp Australia · Autosomal recessive · 2025
Where it sits
Other names
4 names
Resolves to: subcutaneous panniculitis-like T-cell lymphoma
- Also called
- SPTCLsubcutaneous panniculitic T-cell lymphomasubcutaneous panniculitis-like T-cell lymphoma (Alpha/Beta type)subcutaneous panniculitis-like T-cell lymphoma, Alpha/Beta type