风湿性疾病相关噬血细胞综合征诊疗规范  

Recommendations for the diagnosis and treatment of rheumatic diseases-related hemophagocytic syndrome in China

作　　者：王迁 王旖旎[2] 王嫱[3] 张缪佳[3] 孙红胜 刘重阳 王昭[2] 赵岩[1] 中华医学会风湿病学分会 Wang Qian;Wang Yini;Wang Qiang;Zhang Miaojia;Sun Hongsheng;Liu Chongyang;Wang Zhao;Zhao Yan;Chinese Rheumatology Association(Department of Rheumatology and Clinical Immunology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,National Clinical Research Center for Dermatologic and Immunologic Diseases,State Key Laboratory of Complex Sever and Rare Diseases,Key Laboratory of Rheumatology and Clinical Immunology,Ministry of Education,Beijing 100730,China;Department of Hematology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China;Department of Rheumatology and Immunology,the First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China;Department of Rheumatology and Immunology,Shandong Provincial Hospital Affiliated to Shandong First Medical University,Jinan 250021,China;Department of Rheumatology and Clinical Immunology,the Third Affiliated Hospital of Chongqing Medical University,Chongqing 401120,China;不详)

机构地区：[1]中国医学科学院、北京协和医学院、北京协和医院风湿免疫科、国家皮肤与免疫疾病临床医学研究中心、疑难重症及罕见病国家重点实验室、风湿免疫病学教育部重点实验室,北京100730 [2]首都医科大学附属北京友谊医院血液内科,北京100050 [3]南京医科大学第一附属医院风湿免疫科,南京210029 [4]山东第一医科大学附属省立医院风湿免疫科,济南250021 [5]重庆医科大学附属第三医院风湿免疫科,重庆401120 [6]不详

出　　处：《中华内科杂志》2023年第1期23-30,共8页Chinese Journal of Internal Medicine

摘　　要：噬血细胞综合征(HPS)又称噬血细胞性淋巴组织细胞增多症(HLH), 其中继发于风湿性疾病的HLH亦称为巨噬细胞活化综合征(MAS)。HLH是细胞毒T细胞和单核-巨噬细胞系统过度激活, 临床上以持续发热、肝脾肿大、全血细胞减少及骨髓、肝、脾、淋巴结组织出现噬血现象为特征的综合征。MAS是风湿性疾病的高危并发症, 需要多学科密切合作。为提高临床医师对MAS的认识, 规范MAS的临床诊治实践, 中华医学会风湿病学分会组织国内相关专家制定了本规范, 旨在提高MAS的诊治水平, 改善患者预后。Hemophagocytic syndrome(HPS),which is currently named as hemophagocytic lymphohistiocytosis(HLH),is a hyperinflammatory syndrome characterized by persistent fever,hepatosplenomegaly,pancytopenia and hemophagocytosis found in bone marrow,liver,spleen and lymph nodes due to excessive activation of macrophages and cytotoxic T cells.Macrophage activation syndrome(MAS)is a specific form of HLH induced by autoinflammatory/autoimmune disorders which can be life-threatening and requires multiple disciplines.In order to improve clinicians′understanding of MAS and standardize the clinical diagnosis and treatment practice of MAS,the rheumatology branch of Chinese Rheumatology Association organized domestic experts to formulate the diagnosis and treatment standard,in order to improve the diagnosis and treatment level of MAS and improve the prognosis of patients.

关 键 词：噬血细胞综合征 巨噬细胞活化综合征 淋巴组织细胞增多症 嗜血细胞性 自身免疫病 自身炎症性疾病 诊断 治疗 预后 

分 类 号：R593.2[医药卫生—内科学] R55[医药卫生—临床医学]