Recent advances in the treatment and understanding of peripheral T-cell lymphomas (PTCLs) have the potential to improve survival outcomes, according to a review article published in Nature Reviews Cancer.

In their report, Danilo Fiore, PhD, of Weill Cornell Medicine in New York, New York, and colleagues explained that PTCLs represent a diverse class of neoplasms, with PTCL not otherwise specified (PTCL-NOS) being the most abundant subclass and accounting for approximately 30% of all PTCL cases. In addition, patients with PTCL who relapse or progress after initial therapy often have poor survival.

Recurrent translocations, somatic mutations, host-tumor interactions, viral infections, dysregulation of signaling pathways, epigenetic regulatory patterns, and/or metabolism are some of the factors that may be involved in the pathogenesis of PTCL.

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The authors indicated that phenotypic plasticity is widespread even among normal T cells, which can change with environmental stress. This, in addition to the occurrence of mutations in PTCLs, may translate into tumor characteristics that can make it difficult to identify a tumor’s cell of origin. Even so, the authors noted, global gene expression profiling enables insight into a lymphoma’s transcriptional patterns and can help to identify cell type and other features of a tumor. For instance, gene expression profiling has revealed 2 main subgroups into which many cases of PTCL-NOS fall based on transcription factor expression patterns.

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Possible therapy options may be broadening for the treatment of PTCLs. These include targeted therapies and a multitude of immunotherapy options, with potentially more on the horizon as several clinical trials are ongoing across treatment types. The authors also suggested there may be survival gains with use of combination therapies.

“Although still incomplete, the current molecular portraits of the most common PTCLs have uncovered unique and shared properties, as well distinct subgroups with defined clinical outcomes and responses to therapies,” wrote the authors in their report. They also suggested that using therapies in combination may be useful for enhancing survival rates.


Fiore D, Cappelli LV, Broccoli A, Zinzani PL, Chan WC, Inghirami G. Peripheral T cell lymphomas: from the bench to the clinic [published online April 6, 2020]. Nat Rev Cancer. doi: 10.1038/s41568-020-0247-0