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[朗格汉斯细胞组织细胞增多症的免疫微环境:从免疫抑制到靶向治疗]

[Immune microenvironment of Langerhans cell histiocytosis: from immune suppression to targeted therapy].

作者信息

Liu Z Z, Cao X X

机构信息

Department of Medical Oncology, National Cancer Center/National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100021, China Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2025 Jul 14;46(7):673-678. doi: 10.3760/cma.j.cn121090-20241030-00425.

Abstract

Langerhans cell histiocytosis (LCH) is a rare hematologic disorder characterized by the clonal proliferation of neoplastic dendritic cells (DCs), exhibiting both immature and senescent immune phenotypes. The immunosuppressive microenvironment in LCH includes an increased proportion of regulatory T (Treg) cells with inhibitory functions, as well as exhausted CD8(+) T cells and myeloid-derived suppressor cells, which collectively exacerbate immunosuppression and facilitate the immune evasion of tumor DCs. Current therapeutic approaches for LCH are limited by the challenges of relapse and drug resistance. However, emerging strategies that target the senescent phenotype of neoplastic DCs, inhibit Treg cell activity, and reverse T cell exhaustion through immune checkpoint blockade offer promising avenues for the treatment of LCH.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的血液系统疾病,其特征为肿瘤性树突状细胞(DC)的克隆性增殖,呈现不成熟和衰老的免疫表型。LCH中的免疫抑制微环境包括具有抑制功能的调节性T(Treg)细胞比例增加,以及耗竭的CD8(+) T细胞和髓源性抑制细胞,这些共同加剧了免疫抑制并促进肿瘤DC的免疫逃逸。目前LCH的治疗方法受到复发和耐药性挑战的限制。然而,针对肿瘤DC衰老表型、抑制Treg细胞活性以及通过免疫检查点阻断逆转T细胞耗竭的新兴策略为LCH的治疗提供了有前景的途径。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9476/12439734/9ab225c0e7ae/cjh-46-07-673-g001.jpg

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