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蕈样肉芽肿与 Sézary 综合征中产生新免疫治疗方法的细胞与分子异常:文献综合综述

英文原题:Cellular and molecular aberrations generating new immunotherapeutic approaches in mycosis fungoides and Sézary syndrome: a comprehensive review of literature.

查看英文原题

Cellular and molecular aberrations generating new immunotherapeutic approaches in mycosis fungoides and Sézary syndrome: a comprehensive review of literature.

PubMed 2026/07/22(内容时间) Front Immunol Q1 · IF 7(JCR 2025)

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中文摘要

近年来,分子和免疫学分析方面的进展显著加深了对蕈样肉芽肿(MF)和Sézary综合征(SS)的理解,这两种疾病是皮肤T细胞淋巴瘤(CTCL)中最主要的两种亚型。CTCL是一组异质性淋巴系统恶性肿瘤,其特征为具有皮肤趋向性的恶性T细胞克隆性增殖。MF和SS的发病机制似乎由趋同的致癌程序驱动,涉及JAK/STAT、NF-κB、PI3K/AKT/mTOR和MAPK信号通路失调、表观遗传重编程、凋亡抵抗、免疫逃逸以及微环境支持。与此同时,改变的表面表型和趋化因子受体程序塑造了跨皮肤、血液和淋巴结的组织趋向性,而肿瘤微环境则促进肿瘤持续存在和Th2偏斜的免疫极化。这些见解已转化为新型靶向治疗和免疫治疗。本综述总结了目前对MF和SS细胞内在及微环境生物学的认识,并讨论了旨在实现更持久和个性化疾病控制的新兴方法。

展开英文摘要原文

Recent advances in molecular and immunologic profiling have substantially refined the understanding of mycosis fungoides (MF) and S zary syndrome (SS), the two most prominent subtypes of cutaneous T-cell lymphomas (CTCL). CTCL comprise a heterogeneous group of lymphoid malignancies characterized by clonal proliferation of malignant T-cell with cutaneous tropism. The pathogenesis of MF and SS appears to be driven by convergent oncogenic programs involving dysregulated JAK/STAT, NF- B, PI3K/AKT/mTOR, and MAPK signaling, epigenetic reprogramming, apoptosis resistance, immune escape, and microenvironmental support.

In parallel, altered surface phenotypes and chemokine receptor programs shape tissue tropism across skin, blood, and lymph nodes, while the tumor microenvironment promotes tumor persistence and Th2-skewed immune polarization. These insights have translated into novel targeted and immune-based therapies. This review summarizes current insights into the cell-intrinsic and microenvironmental biology of MF and SS and discusses emerging approaches aimed at achieving more durable and personalized disease control.

论文信息

作者
Flechtenmacher AS、Melchers S、Nicolay JP
单位
Department of Dermatology, Venerology and Allergology, University Medical Center Mannheim/University of Heidelberg, Mannheim, Germany.Germany
文献类型
综述
期刊
Frontiers in immunology2026
原文标识
PubMed 42558889 · DOI 10.3389/fimmu.2026.1899436