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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Main Authors: Fanny Drieux, François Lemonnier, Philippe Gaulard
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-06-01
Series:Frontiers in Oncology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fonc.2023.1202964/full
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author Fanny Drieux
François Lemonnier
François Lemonnier
Philippe Gaulard
Philippe Gaulard
author_facet Fanny Drieux
François Lemonnier
François Lemonnier
Philippe Gaulard
Philippe Gaulard
author_sort Fanny Drieux
collection DOAJ
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 doaj.art-a828d02c1c9741efa5e29277c24500cf2023-06-23T17:18:43ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2023-06-011310.3389/fonc.2023.12029641202964How molecular advances may improve the diagnosis and management of PTCL patientsFanny Drieux0François Lemonnier1François Lemonnier2Philippe Gaulard3Philippe Gaulard4Service d’Anatomie et de Cytologie Pathologiques, INSERM U1245, Centre Henri Becquerel, Rouen, FranceUnité hémopathies Lymphoïdes, Hôpitaux Universitaires Henri Mondor, Assistance Publique des Hôpitaux de Paris, Créteil, FranceInstitut Mondor de Recherche Biomédicale, INSERM U955, Université Paris Est Créteil, Créteil, FranceInstitut Mondor de Recherche Biomédicale, INSERM U955, Université Paris Est Créteil, Créteil, FranceDépartement de Pathologie, Hôpitaux Universitaires Henri Mondor, Assistance Publique des Hôpitaux de Paris, Créteil, FrancePeripheral 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.https://www.frontiersin.org/articles/10.3389/fonc.2023.1202964/fullperipheral T-cell lymphomamolecular diagnosisoncogenesisdiagnosistargeted therapy
spellingShingle Fanny Drieux
François Lemonnier
François Lemonnier
Philippe Gaulard
Philippe Gaulard
How molecular advances may improve the diagnosis and management of PTCL patients
Frontiers in Oncology
peripheral T-cell lymphoma
molecular diagnosis
oncogenesis
diagnosis
targeted therapy
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 peripheral T-cell lymphoma
molecular diagnosis
oncogenesis
diagnosis
targeted therapy
url https://www.frontiersin.org/articles/10.3389/fonc.2023.1202964/full
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