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SOHO 最新进展与后续问题 | 外周 T 细胞淋巴瘤:当前格局与新兴问题

英文原题:SOHO State of the Art Updates and Next Questions | Peripheral T-Cell Lymphoma: Current Landscape and Emerging Questions.

PubMed 2026/02/18(内容时间) Clin Lymphoma Myeloma Leuk Q1 · IF 4.1(JCR 2025)

研究概要

外周T细胞淋巴瘤(PTCLs)是一组罕见的、异质性的成熟T细胞和NK细胞肿瘤,约占所有非霍奇金淋巴瘤的10%至15%。

中文摘要

外周T细胞淋巴瘤(PTCLs)是一组罕见的、异质性的成熟T细胞和NK细胞肿瘤,约占所有非霍奇金淋巴瘤的10%至15%。尽管在疾病分类和生物学方面取得了进展,但除间变性淋巴瘤激酶(ALK)阳性的间变性大细胞淋巴瘤(ALCL)外,大多数亚型的临床结局仍然不佳。本综述综合了目前对结内PTCL生物学的认识——包括T细胞滤泡辅助(TFH)来源的淋巴瘤、PTCL-NOS和系统性ALCL。分子谱分析的整合加深了我们对疾病异质性的理解,识别出反复出现的表观遗传和信号通路改变,这些改变如今为合理的靶向策略提供了依据。以维布妥昔单抗为基础的方案重塑了CD30阳性疾病的一线治疗,而正在进行的研究正在探索新型疗法在PTCL谱系中的作用,包括表观遗传调节剂、磷脂酰肌醇3-激酶(PI3K)和JAK抑制剂以及免疫基础疗法。未来的进展将取决于生物标志物驱动的临床试验、精细化的患者选择,以及整合基因组和免疫特征以实现个体化治疗。

展开英文摘要原文

Peripheral T-cell lymphomas (PTCLs) comprise a rare, heterogeneous group of mature T-cell and NK-cell neoplasms, representing approximately 10% to 15% of all non-Hodgkin lymphomas. Despite advances in disease classification and biology, clinical outcomes remain poor for most subtypes other than anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL). This review synthesizes current understanding of nodal PTCL biology-including T-cell follicular helper (TFH)-derived lymphomas, PTCL-NOS, and systemic ALCL. The integration of molecular profiling has deepened our understanding of disease heterogeneity, identifying recurrent epigenetic and signaling pathway alterations that now inform rational, targeted strategies. Brentuximab vedotin-based regimens have reshaped frontline therapy for CD30-positive disease, while ongoing studies are exploring the role of novel therapies including epigenetic modulators, phosphatidylinositol 3-kinase (PI3K) and JAK inhibitors, and immune-based therapies across the PTCL spectrum. Future progress will depend on biomarker-driven clinical trials, refined patient selection, and the incorporation of genomic and immune signatures to personalize therapy.

论文信息

作者
Tolu SS、Pro B
第一作者单位
Department of Medicine, Columbia University Irving Medical Center, New York, NY.United States
通讯作者单位
Department of Medicine, Columbia University Irving Medical Center, New York, NY. Electronic address: bp2655@cumc.columbia.edu.United States
文献类型
综述
期刊
Clinical lymphoma, myeloma & leukemia2026 Jun
原文标识
PubMed 42020275 · DOI 10.1016/j.clml.2026.02.007