成人T细胞白血病/淋巴瘤诊断与治疗中国专家共识(2024年版)  

Chinese expert consensus on the diagnosis and treatment of adult T-cell leukemia/lymphoma(2024)

作　　者：中国医师协会血液科医师分会 中国医疗保健国际交流促进会血液学分会 王少元[2] 黄晓军[3] 吴德沛[4] Hematology Physicians Committee of Chinese Medical Doctor Association;China International Exchange and Promotive Association for Medical and Healthcare,Society of Hematology;Wang Shaoyuan;Huang Xiaojun;Wu Depei(不详;Fujian Medical University Union Hospital,Fujian Institute of Hematology,Fujian Provincial Key Laboratory on Hematology,Fuzhou 350001,China;Peking University People′s Hospital,Peking University Institute of Hematology,National Clinical Research Center for Hematologic Diseases,Beijing 100044,China;the First Affiliated Hospital of Soochow University,Jiangsu Institute of Hematology,National Clinical Research Center for Hematologic Diseases,Suzhou 215006,China)

机构地区：[1]不详 [2]福建医科大学附属协和医院、福建省血液病研究所福建省血液病重点实验室,福州350001 [3]北京大学人民医院、北京大学血液病研究所、国家血液系统疾病临床医学研究中心,北京100044 [4]苏州大学附属第一医院、江苏省血液研究所国家血液系统疾病临床医学研究中心,苏州215006

出　　处：《中华内科杂志》2024年第12期1196-1204,共9页Chinese Journal of Internal Medicine

基　　金：国家自然科学基金(82070157,82000210);国家重点研发计划(2022YFC2502700);国家临床重点专科建设项目(闽卫医政2021-76号);福建省恶性血液病临床医学研究中心项目(2020Y2006)。

摘　　要：成人T细胞白血病/淋巴瘤(ATL)是一种少见且独特的T淋巴细胞增殖性肿瘤,发病与Ⅰ型人类嗜T淋巴细胞病毒(HTLV-1)感染密切相关。ATL临床特征异质性强,耐药常见,机会性感染频繁,预后差,是一种极具挑战性的疾病。由于ATL发病例数较少,国内对该疾病的认识尚未统一,诊疗尚不规范。为加强我国临床医师对ATL的认知,中国血液学专家结合国内外的最新研究进展,讨论并制订了《成人T细胞白血病/淋巴瘤诊断与治疗中国专家共识(2024年版)》。本共识提出中国ATL最新的诊断标准与分型标准,并简述了HTLV-1在中国沿海地区的感染情况,国内ATL的流行趋势、发病机制、临床表现、治疗策略等方面的最新证据和建议,旨在规范ATL的临床诊治,改善ATL患者的总体生存。Adult T-cell leukemia/lymphoma(ATL),a rare and unique proliferative tumor of T lymphocytes,is associated with human T-cell leukemia virus type 1(HTLV-1)infection.Due to its diverse clinical features,multidrug resistance of malignant cells,frequent opportunistic infection,and poor prognosis,ATL remains a clinical challenge.A unified understanding of ATL is currently lacking,and its diagnosis and treatment are not standardized in China due to the small number of cases.Therefore,to strengthen our understanding of ATL,hematology experts have formulated the"Chinese expert consensus on the diagnosis and treatment of adult T-cell leukemia/lymphoma(2024)"based on the latest research progress at home and abroad.This consensus proposes the latest diagnostic and subtype criteria for ATL in China.Furthermore,it briefly describes the infection situation of HTLV-1 in coastal areas of China,the epidemic trends in China,and the latest evidence and recommendations regarding the pathogenesis,clinical manifestations,and treatment strategies,with the aim to standardize the clinical diagnosis and treatment of ATL and ultimately improve the overall survival of patients with ATL.

关 键 词：成人T细胞白血病/淋巴瘤 人类嗜T淋巴细胞病毒1型 诊断 分型 鉴别诊断 治疗 

分 类 号：R733.7[医药卫生—肿瘤]