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外周T细胞淋巴瘤:下一步何去何从?

Peripheral T-Cell Lymphoma: What's Next?

作者信息

Yoon Sang Eun, Kim Won Seog

机构信息

Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.

出版信息

Hematol Oncol. 2025 Jun;43 Suppl 2(Suppl 2):e70069. doi: 10.1002/hon.70069.

Abstract

Peripheral T-cell lymphoma (PTCL) is a rare and heterogeneous group of diseases, with over 30 subtypes according to the International Consensus Classification of Mature Lymphoid Neoplasms (ICC) and World Health Organization Classification of Hematolymphoid Tumors (WHO-HEM) 2022. The classification complexity reflects the underlying genetic and biological diversity of PTCL. For decades, distinct PTCL subtypes have been uniformly treated with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or CHOP-like regimens originally developed for mainly B-cell lymphoma. Attempts to improve frontline CHOP-plus strategies have failed mainly due to toxicities and lack of biological rationale. Only the ECHELON-2 trial succeeded as more than 70% of patients had anaplastic large cell lymphoma (ALCL), where brentuximab vedotin (BV) is most effective. Looking ahead to 2025 and beyond, future treatment strategies for PTCL should be guided by a deeper understanding of its underlying biology rather than relying on empirical extrapolations from other lymphomas.

摘要

外周T细胞淋巴瘤(PTCL)是一组罕见且异质性的疾病,根据国际成熟淋巴细胞肿瘤共识分类(ICC)和世界卫生组织血液淋巴肿瘤分类(WHO-HEM)2022,有30多种亚型。分类的复杂性反映了PTCL潜在的遗传和生物学多样性。几十年来,不同的PTCL亚型一直统一采用CHOP(环磷酰胺、阿霉素、长春新碱和泼尼松)或最初主要为B细胞淋巴瘤开发的类似CHOP方案进行治疗。改善一线CHOP加方案的尝试主要因毒性和缺乏生物学依据而失败。只有ECHELON-2试验取得成功,因为超过70%的患者患有间变性大细胞淋巴瘤(ALCL),而本妥昔单抗(BV)在该疾病中最为有效。展望2025年及以后,PTCL的未来治疗策略应以对其潜在生物学的更深入理解为指导,而不是依赖于从其他淋巴瘤进行的经验性推断。

相似文献

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Peripheral T-Cell Lymphoma: What's Next?外周T细胞淋巴瘤:下一步何去何从?
Hematol Oncol. 2025 Jun;43 Suppl 2(Suppl 2):e70069. doi: 10.1002/hon.70069.
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Frontline Management of Nodal Peripheral T-Cell Lymphomas.结外边缘区 T 细胞淋巴瘤的一线治疗。
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