Durgin Joseph S, Weiner David M, Wysocka Maria, Rook Alain H
Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
J Am Acad Dermatol. 2021 Mar;84(3):587-595. doi: 10.1016/j.jaad.2020.12.027. Epub 2020 Dec 22.
Cutaneous T cell lymphomas (CTCLs) are malignancies of skin-trafficking T cells. Patients with advanced CTCL manifest immune dysfunction that predisposes to infection and suppresses the antitumor immune response. Therapies that stimulate immunity have produced superior progression-free survival compared with conventional chemotherapy, reinforcing the importance of addressing the immune deficient state in the care of patients with CTCL. Recent research has better defined the pathogenesis of these immune deficits, explaining the mechanisms of disease progression and revealing potential therapeutic targets. The features of the malignant cell in mycosis fungoides and Sézary syndrome are now significantly better understood, including the T helper 2 cell phenotype, regulatory T cell cytokine production, immune checkpoint molecule expression, chemokine receptors, and interactions with the microenvironment. The updated model of CTCL immunopathogenesis provides understanding into clinical progression and therapeutic response.
皮肤T细胞淋巴瘤（CTCL）是皮肤游走性T细胞的恶性肿瘤。晚期CTCL患者表现出免疫功能障碍，易发生感染并抑制抗肿瘤免疫反应。与传统化疗相比，刺激免疫的疗法已产生了更好的无进展生存期，这强化了在CTCL患者护理中解决免疫缺陷状态的重要性。最近的研究更好地明确了这些免疫缺陷的发病机制，解释了疾病进展的机制并揭示了潜在的治疗靶点。蕈样肉芽肿和Sezary综合征中恶性细胞的特征现在已得到了显著更好的理解，包括辅助性T细胞2细胞表型、调节性T细胞细胞因子产生、免疫检查点分子表达、趋化因子受体以及与微环境的相互作用。CTCL免疫发病机制的更新模型有助于理解临床进展和治疗反应。
