Drieux Fanny, Lemonnier François, Gaulard Philippe
Service d'Anatomie et de Cytologie Pathologiques, INSERM U1245, Centre Henri Becquerel, Rouen, France.
Unité hémopathies Lymphoïdes, Hôpitaux Universitaires Henri Mondor, Assistance Publique des Hôpitaux de Paris, Créteil, France.
Front Oncol. 2023 Jun 23;13:1202964. doi: 10.3389/fonc.2023.1202964. eCollection 2023.
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.
外周T细胞淋巴瘤（PTCL）由30多种罕见的异质性实体组成，占成人非霍奇金淋巴瘤的10%至15%。尽管其诊断仍主要基于临床、病理和表型特征，但分子研究有助于更好地理解其中涉及的致癌机制，并在最近更新的分类中对许多PTCL实体进行了细化。尽管经过多年临床试验，但对于大多数实体（5年总生存率<30%），基于蒽环类药物的多药化疗方案的当前传统疗法预后仍然很差。最近使用的新靶向疗法对复发/难治性患者似乎很有前景，例如T滤泡辅助（TFH）PTCL中的去甲基化剂。然而，需要进一步研究来评估这些药物在一线治疗中的合理组合。在本综述中，我们将总结主要PTCL实体的致癌事件，并报告导致新疗法开发的分子靶点。我们还将讨论创新的高通量技术的发展，这些技术有助于PTCL患者组织病理学诊断和管理的常规工作流程。
