Information on SPTCL
Name: T-cell lymphoma, subcutaneous panniculitis-like | Acronym: SPTCL
Alt. names: susceptibility to the development of subcutaneous panniculitis-like T-cell lymphoma
Gene: HAVCR2 | MOI: Autosomal recessive | Mechanism of action:
No. of cases in DB: 0 | First reported in: 2018
Last updated on: 2023-02-28 by
Description
Individuals have been described as being susceptible to form of T-cell non-Hodgkin lymphoma, and awareness may allow early surveillance, diagnosis, and management (immunosuppressive and chemotherapeutic treatment has been reported as effective); HSCT has been reported as curative. Refs. PMIDs: 30374066; 30792187
Management
Description of management option has not been reviewed yet.
Please mind that full curation of this condition has not started yet. Please contact us if you want to volunteer.
Summary of clinical findings
[Considering only Definitive and Possible cases]
| Rank | Clinical phenotype | Present | Absent | Unreported |
|---|---|---|---|---|
| 1 | Panniculitis |
16 (99.9%) | 0 (0.0%) | 0 (0.0%) |
| 2 | Subcutaneous panniculitis-like T-cell lymphoma |
16 (99.9%) | 0 (0.0%) | 0 (0.0%) |
| 3 | Juvenile onset |
7 (43.8%) | 0 (0.0%) | 9 (56.3%) |
| 4 | Young adult onset |
4 (25.0%) | 0 (0.0%) | 12 (75.0%) |
| 5 | Late onset |
2 (12.5%) | 0 (0.0%) | 14 (87.5%) |
| 6 | Middle age onset |
2 (12.5%) | 0 (0.0%) | 14 (87.5%) |
| 7 | Childhood onset |
1 (6.3%) | 0 (0.0%) | 15 (93.8%) |
Age of onset
distribution
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Summary of treatment outcomes
[Considering only Definitive and Possible cases]
| Treatment ⓘ | Responses & clinical indications |
|---|
Please mind that full curation of this condition has not started yet. Please contact us if you want to volunteer.
0 reported cases added to GenIA
| SubjectID | Sex | Fam.ID | AD | AFM | Validity | Country | Population | Reference & Pub.code |
|---|
AD: Age at genetic diagnosis; AFM: age at first manifestation; PMID: PubMed ID; GRID: GenIA reference ID (ref. not in PubMed).