皮肤T细胞淋巴瘤的诊治进展  

Advances in the diagnosis and treatment of cutaneous T-cell lymphoma

作　　者：晋红中[1] 唐珂韵 JIN Hongzhong;TANG Keyun(State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Science and Peking Union Medical College,Beijing 100005,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院,疑难重症及罕见病国家重点实验室,国家皮肤与免疫疾病临床医学研究中心,北京100005

出　　处：《皮肤性病诊疗学杂志》2023年第5期391-401,共11页Journal of Diagnosis and Therapy on Dermato-venereology

基　　金：中央高水平医院临床科研业务费项目(2022-PUMCH-B-092);中国医学科学院医学与健康科技创新工程项目(2021-I2M-1-059);国家自然科学基金面上项目(82073450)。

摘　　要：皮肤T细胞淋巴瘤是一组异质性的非霍奇金淋巴瘤,重要代表有蕈样肉芽肿(MF)和Sezary综合征(SS)。MF/SS的诊断主要依靠临床表现、皮肤组织病理、免疫组织化学染色、外周血流式细胞学分析、克隆性T细胞受体基因重排或其他克隆性检测。MF/SS的治疗需根据疾病分期进行阶梯性治疗,分为皮肤定向治疗和系统治疗。近年来,国内外专家针对MF/SS的诊疗提出新的见解和诊疗方案,且随着分子诊断方法、新型生物标志物、皮肤影像学、人工智能等领域的蓬勃发展,多种新型靶向及生物免疫治疗方法不断涌现。本文主要综述MF/SS的诊治进展,期待为临床应用提供新思路。Mycosis fungoides(MF)and Sezary syndrome(SS)are important representatives of the heterogeneous group of cutaneous T-cell lymphoma.The diagnosis of MF/SS is based on the clinical,histopathologic and immunohistochemical findings,flow cytometric analysis of peripheral blood and T-cell receptor gene rearrangement,as well as other genetic analysis.The treatments for MF/SS are always stage-oriented and are classified into topical(skin-directed)and systemic therapies.Chinese and international experts in this field have updated recommendations and guidelines for the diagnosis and treatment of MF/SS.There are tremendous evolvements in the diagnosis of MF/SS,including molecular diagnostic methods,molecular biomarkers,dermoscopic imaging and artificial intelligence.Novel disease-specific targeted therapy,biologic therapy and immunomodulatory drugs have emerged.This review summarizes advances in the diagnosis and treatments of MF/SS,which may provide a reference for clinical practice and future research.

关 键 词：皮肤淋巴瘤 T细胞 蕈样肉芽肿 SEZARY综合征 诊断 治疗 

分 类 号：R739.5[医药卫生—肿瘤]