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How molecular advances may improve the diagnosis and management of PTCL patients

Peripheral T-cell lymphomas (PTCL) comprised more than 30 rare heterogeneous entities, representing 10 to 15% of adult non-Hodgkin lymphomas. Although their diagnosis is still mainly based on clinical, pathological, and phenotypic features, molecular studies have allowed for a better understanding o...

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Autores principales: Drieux, Fanny, Lemonnier, François, Gaulard, Philippe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10328093/
https://www.ncbi.nlm.nih.gov/pubmed/37427095
http://dx.doi.org/10.3389/fonc.2023.1202964
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author Drieux, Fanny
Lemonnier, François
Gaulard, Philippe
author_facet Drieux, Fanny
Lemonnier, François
Gaulard, Philippe
author_sort Drieux, Fanny
collection PubMed
description Peripheral T-cell lymphomas (PTCL) comprised more than 30 rare heterogeneous entities, representing 10 to 15% of adult non-Hodgkin lymphomas. Although their diagnosis is still mainly based on clinical, pathological, and phenotypic features, molecular studies have allowed for a better understanding of the oncogenic mechanisms involved and the refinement of many PTCL entities in the recently updated classifications. The prognosis remains poor for most entities (5-year overall survival < 30%), with current conventional therapies based on anthracyclin-based polychemotherapy regimen, despite many years of clinical trials. The recent use of new targeted therapies appears to be promising for relapsed/refractory patients, such as demethylating agents in T-follicular helper (TFH) PTCL. However further studies are needed to evaluate the proper combination of these drugs in the setting of front-line therapy. In this review, we will summarize the oncogenic events for the main PTCL entities and report the molecular targets that have led to the development of new therapies. We will also discuss the development of innovative high throughput technologies that aid the routine workflow for the histopathological diagnosis and management of PTCL patients.
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spelling pubmed-103280932023-07-08 How molecular advances may improve the diagnosis and management of PTCL patients Drieux, Fanny Lemonnier, François Gaulard, Philippe Front Oncol Oncology Peripheral T-cell lymphomas (PTCL) comprised more than 30 rare heterogeneous entities, representing 10 to 15% of adult non-Hodgkin lymphomas. Although their diagnosis is still mainly based on clinical, pathological, and phenotypic features, molecular studies have allowed for a better understanding of the oncogenic mechanisms involved and the refinement of many PTCL entities in the recently updated classifications. The prognosis remains poor for most entities (5-year overall survival < 30%), with current conventional therapies based on anthracyclin-based polychemotherapy regimen, despite many years of clinical trials. The recent use of new targeted therapies appears to be promising for relapsed/refractory patients, such as demethylating agents in T-follicular helper (TFH) PTCL. However further studies are needed to evaluate the proper combination of these drugs in the setting of front-line therapy. In this review, we will summarize the oncogenic events for the main PTCL entities and report the molecular targets that have led to the development of new therapies. We will also discuss the development of innovative high throughput technologies that aid the routine workflow for the histopathological diagnosis and management of PTCL patients. Frontiers Media S.A. 2023-06-23 /pmc/articles/PMC10328093/ /pubmed/37427095 http://dx.doi.org/10.3389/fonc.2023.1202964 Text en Copyright © 2023 Drieux, Lemonnier and Gaulard https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Drieux, Fanny
Lemonnier, François
Gaulard, Philippe
How molecular advances may improve the diagnosis and management of PTCL patients
title How molecular advances may improve the diagnosis and management of PTCL patients
title_full How molecular advances may improve the diagnosis and management of PTCL patients
title_fullStr How molecular advances may improve the diagnosis and management of PTCL patients
title_full_unstemmed How molecular advances may improve the diagnosis and management of PTCL patients
title_short How molecular advances may improve the diagnosis and management of PTCL patients
title_sort how molecular advances may improve the diagnosis and management of ptcl patients
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10328093/
https://www.ncbi.nlm.nih.gov/pubmed/37427095
http://dx.doi.org/10.3389/fonc.2023.1202964
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