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ptcl
Peripheral T-cell Lymphoma
PTCLs are aggressive T/NK-cell lymphomas (~10-15% of NHL); major subtypes PTCL-NOS (~25%), AITL (~20%), ALK+ ALCL (~7%), ALK- ALCL (~8%); AITL driven by TET2+DNMT3A+RHOA G17V; brentuximab vedotin+CHP is standard for CD30+ PTCL (ECHELON-2); 5-year OS ~30-50%.
Entry Metadata
| Field | Value |
|---|---|
| ID | ptcl |
| Name | Peripheral T-cell Lymphoma |
| Status | draft |
| Last reviewed | 2026-06-06 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Horwitz S, O'Connor OA, Pro B, et al. Brentuximab vedotin with chemotherapy for CD30-positive peripheral T-cell lymphoma (ECHELON-2): a global, double-blind, randomised, phase 3 trial. Lancet. 2019;393(10168):229-240. · PubMed 30522922
- Palomero T, Couronné L, Khiabanian H, et al. Recurrent mutations in epigenetic regulators, RHOA and FYN kinase in peripheral T cell lymphomas. Nat Genet. 2014;46(2):166-170. · PubMed 24413734