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结内外周 T 细胞淋巴瘤的过去、现在与未来治疗策略

英文原题:Past, present and future therapeutic approaches in nodal peripheral T-cell lymphomas.

PubMed 2023/12/01(内容时间) Haematologica Q1 · IF 8.2(JCR 2025)

研究概要

外周 T 细胞淋巴瘤(PTCL)涵盖 30 多种不同实体,尽管它们均起源于胸腺后 T 细胞或 NK 细胞,但各亚型的疾病生物学和基因组特征非常多样。

中文摘要

外周T细胞淋巴瘤(PTCL)包括30余种不同疾病;尽管它们均来源于胸腺后T细胞或NK细胞,但不同亚型的疾病生物学和基因组图谱差异很大。在西方人群中,淋巴结型PTCL是临床上最常见的类型。尽管在阐明其基础生物学及推进治疗方面已有重要进展,但对疾病的认识仍有明显不足,临床实践中最佳方案仍存在争议。CHOP(环磷酰胺、多柔比星、长春新碱和泼尼松)方案化疗仍是“标准”治疗。在CD30阳性PTCL中,CHP(环磷酰胺、多柔比星和泼尼松)联合维布妥昔单抗(BV)代表新的治疗模式,但其在非间变性大细胞淋巴瘤亚型中的获益尚不确定。鉴于复发风险较高,除ALK阳性间变性大细胞淋巴瘤外,淋巴结型PTCL患者可考虑巩固性自体干细胞移植;但由于缺乏随机对照试验,临床实践存在差异。除CHP-BV外,多数研究集中于在CHOP基础上加用新药。部分新型联合治疗显示完全缓解率较高,因此也正在一线治疗中进行试验,尤其是具有明确表观遗传调节疗法敏感性的滤泡辅助性T细胞淋巴瘤。复发/难治性疾病的治疗很好地体现了这一趋势:正在针对特定亚型或依据其基础生物学开发合理联合方案。总之,淋巴结型PTCL的管理终于进入更加个体化的时代。

展开英文摘要原文

Peripheral T-cell lymphomas (PTCL) encompass over 30 different entities and although they share post-thymic T- or NK-cell derivation, the disease biology and genomic landscape are very diverse across subtypes. In Western populations, nodal PTCL are the most frequently encountered entities in clinical practice and although important achievements have been made in deciphering the underlying biology and in therapeutic advances, there are still large gaps in disease understanding and clinical scenarios in which controversy over best practice continues. CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone)- based chemotherapy continues to be the 'standard' treatment, with the addition of brentuximab vedotin (BV) in the combination CHP (cyclosphosphamide, doxorubicin, prednisone)-BV representing a new treatment paradigm in CD30+ PTCL although its benefit is less certain in the non-anaplastic large cell lymphoma subtypes. Given the high risk of relapse, consolidative autologous stem cell transplant is considered in nodal PTCL, outside of ALK-positive anaplastic large cell lymphoma; however, in the absence of a randomized controlled trials, practices vary. Beyond CHP-BV, most study activity has focused on adding a novel agent to CHOP (i.e., CHOP + drug X). However, with high complete remission rates observed with some novel therapy combinations, these regimens are being tested in the front-line setting, with a particular rationale in follicular helper T-cell lymphomas which have a clear sensitivity to epigenetic modifying therapies. This is well exemplified in the relapsed/refractory setting in which rational combination therapies are being developed for specific subtypes or guided by underlying biology. Taken together, we have finally moved into an era of a more personalized approach to the management of nodal PTCL.

论文信息

作者
Ngu HS、Savage KJ
第一作者单位
Center for Lymphoid Cancer, Division of Medical Oncology BC Cancer and the University of British Columbia, British Columbia, Vancouver.Canada
通讯作者单位
Center for Lymphoid Cancer, Division of Medical Oncology BC Cancer and the University of British Columbia, British Columbia, Vancouver. ksavage@bccancer.bc.ca.Canada
文献类型
非美国政府资助研究 · 评论
期刊
Haematologica2023 Dec 1
原文标识
PubMed 38037799 · DOI 10.3324/haematol.2021.280275