Peripheral T-Cell Lymphoma, NOS (PTCL)

Overview

Peripheral T-Cell lymphoma, NOS is a Mature T and NK Neoplasm (parent MTNN).

mtnn_msk_2022: 132 adults with nodal PTCL (PTCL-NOS, AITL, PTCL-TFH, ALK+/− ALCL) plus EATL and MEITL, treated at MSK 2015–2020 with curative-intent CHOP-based chemotherapy and profiled on MSK-IMPACT PMID:37078708.
Histology mix: AITL 47%, PTCL-NOS 27%, ALK− ALCL 11%, PTCL-TFH 7%, ALK+ ALCL 5%, MEITL 3% PMID:37078708.

Most frequently mutated genes: TET2 52%, RHOA 30%, DNMT3A 19%, TP53 16%, IDH2 11% PMID:37078708.
Most common CNAs: TP53 deletions 7% and CDKN2A deletions 7% PMID:37078708.
TP53 mutations (HR 3.1, 95% CI 1.4–6.8, P=.005) and TP53/17p deletions (HR 4.1, 95% CI 1.1–15.0, P=.03) independently predicted inferior PFS on multivariate analysis PMID:37078708.
Median PFS: 4.5 months for TP53-mutated PTCL (n=21) vs 10.5 months for TP53-wildtype (n=111) PMID:37078708.
CDKN2A deletion correlated with inferior OS (HR 12.1; 95% CI 2.8–52.0; P<.001); median OS 17.6 vs 56.7 months PMID:37078708.
No TP53 aberrancy correlated with inferior OS PMID:37078708.
RHOA G17V (dominant-negative, loss-of-function) is recurrent across peripheral T-cell lymphomas including AITL (~67% of AITL cases) and other PTCL subtypes; functional yeast modelling separates RHOA GOF and LOF mutants into distinct morphological classes PMID:24816253

Curative-intent CHOP-based chemotherapy regimens: CHOEP/EPOCH 45%, CHOP 30%, BV-CH(E)P 12%, CHOP + novel agent 13% PMID:37078708.
TP53 mutation status may warrant consideration of alternative induction/consolidation strategies and stratification in first-line randomized trials PMID:37078708.

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