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Mature systemic T-cell lymphomas (MTCLs) represent relatively rare but extremely heterogeneous subgroup of lymphoproliferative disorders. MTCLs have much worse outcomes than B-cell lymphomas. Although gene expression profiling, next-generation sequencing, and transgenic mouse modeling have better elucidated ...

Mature systemic T-cell lymphomas (MTCLs) represent relatively rare but extremely heterogeneous subgroup of lymphoproliferative disorders. MTCLs have much worse outcomes than B-cell lymphomas. Although gene expression profiling, next-generation sequencing, and transgenic mouse modeling have better elucidated the pathogenic mechanism of T-cell lymphoma biology, genomic-based stratifications have not yet entered into routine clinical practice. The biological significance of many genetic/epigenetic alterations are becoming better understood. Altogether these advances will continue to refine diagnostic criteria, improve prognostication, and identify novel therapeutic targets, resulting in improved outcomes for patients with MTCLs. Moreover, MTCLs are characterized by immense differences of particular subtype distribution, which indicates the significant influence of ethnic and/or geographical immune backgrounds.

There are over 27 different subtypes of MTCLs and we are now beginning to understand the differences between the various subtypes beyond histologic variations. The “one chemotherapy fits for all” approach, which has been generally used (with some exceptions) in MTCLs, is not acceptable. Up to now, no relevant molecular marker usable in clinical practice was identified with the exception of ALK and CD30. With growing knowledge about specific properties of malignant T-cells irrespective of their histologic diagnosis, the approaches including antibodies, inhibitors of cytokine-receptor interactions, and kinase inhibitors may revolutionize therapy for MTCLs. Besides, the targetable epigenetic alterations are observed in all MTCLs, but specific subtypes demonstrate enrichment for particular recurrent alterations targeting specific genes. The role of the tumoral microenvironment and immune crosstalk, which is so popular in Hodgkin and B-cell lymphomas, has rarely been studied. The use of checkpoint inhibitors in the treatment of MTCLs still remains controversial.

In this Research Topic we would like to summarize recent knowledge and new perspectives about mainly systemic T-cell lymphomas coming from potentially targetable molecular changes of tumoral cells to the immune microenvironment. We welcome Original Articles, Reviews, Systematic Reviews, Clinical Trials and Case Reports focused on the following themes:

1) preclinical studies of genomic and epigenomic changes related to T-cell lymphoma pathogenesis

2) preclinical or clinical studies investigating the immune microenvironment and its role in the development and biology of T-cell lymphomas

3) preclinical or clinical studies or experiences with targeted drugs (kinase inhibitors, epigenetic modifiers, etc)

4) preclinical and clinical research of ALK, CD30 in pathogenesis and biology of T-cell lymphomas

6) novel prognosticators based on lymphoma biology

Please note: manuscripts consisting solely of bioinformatics, computational analysis, or predictions of public databases which are not accompanied by validation (independent cohort or biological validation in vitro or in vivo) will not be accepted in any of the sections of Frontiers in Oncology.

Keywords: T-cell lymphoma, microenvironment, epigenetic, gene expression, CD30, ALK, prognosis


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