Liu Zhaorui, Wu Xuesong, Hwang Sam T, Liu Jie
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.
Department of Dermatology, School of Medicine, University of California Davis, Sacramento, CA, United States.
Ann Dermatol. 2021 Dec;33(6):487-496. doi: 10.5021/ad.2021.33.6.487. Epub 2021 Nov 4.
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphomas (CTCLs). Most cases of MF display an indolent course during its early stage. However, in some patients, it can progress to the tumor stage with potential systematic involvement and a poor prognosis. SS is defined as an erythrodermic CTCL with leukemic involvements. The pathogenesis of MF and SS is still not fully understood, but recent data have found that the development of MF and SS is related to genetic alterations and possibly to environmental influences. In CTCL, many components interacting with tumor cells, such as tumor-associated macrophages, fibroblasts, dendritic cells, mast cells, and myeloid-derived suppressor cells, as well as with chemokines, cytokines and other key players, establish the tumor microenvironment (TME). In turn, the TME regulates tumor cell migration and proliferation directly and indirectly and may play a critical role in the progression of MF and SS. The TME of MF and SS appear to show features of a Th2 phenotype, thus dampening tumor-related immune responses. Recently, several studies have been published on the immunological characteristics of MF and SS, but a full understanding of the CTCL-related TME remains to be determined. This review focuses on the role of the TME in MF and SS, aiming to further demonstrate the pathogenesis of the disease and to provide new ideas for potential treatments targeted at the microenvironment components of the tumor.
蕈样肉芽肿（MF）和塞扎里综合征（SS）是皮肤T细胞淋巴瘤（CTCL）最常见的亚型。大多数MF病例在早期呈惰性病程。然而，在一些患者中，它可进展至肿瘤期，伴有潜在的全身受累且预后不良。SS被定义为伴有白血病浸润的红皮病型CTCL。MF和SS的发病机制仍未完全阐明，但最近的数据发现，MF和SS的发生与基因改变以及可能的环境影响有关。在CTCL中，许多与肿瘤细胞相互作用的成分，如肿瘤相关巨噬细胞、成纤维细胞、树突状细胞、肥大细胞和髓系来源的抑制细胞，以及趋化因子、细胞因子和其他关键因子，共同构成了肿瘤微环境（TME）。反过来，TME直接或间接调节肿瘤细胞的迁移和增殖，并可能在MF和SS的进展中起关键作用。MF和SS的TME似乎表现出Th2表型的特征，从而抑制肿瘤相关的免疫反应。最近，已有多项关于MF和SS免疫特征的研究发表，但对CTCL相关TME的全面了解仍有待确定。本综述重点关注TME在MF和SS中的作用，旨在进一步阐明该疾病的发病机制，并为针对肿瘤微环境成分的潜在治疗提供新思路。
