Wang Q, Wang Y N, Wang Q, Zhang M J, Sun H S, Liu C Y, Wang Z, Zhao Y
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.
Zhonghua Nei Ke Za Zhi. 2023 Jan 1;62(1):23-30. doi: 10.3760/cma.j.cn112138-20221010-00749.
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.
噬血细胞综合征（HPS），现称为噬血细胞性淋巴组织细胞增生症（HLH），是一种高炎症综合征，其特征为持续发热、肝脾肿大、全血细胞减少以及在骨髓、肝脏、脾脏和淋巴结中发现由于巨噬细胞和细胞毒性T细胞过度激活导致的噬血细胞现象。巨噬细胞活化综合征（MAS）是由自身炎症/自身免疫性疾病诱发的HLH的一种特殊形式，可危及生命且需要多学科协作。为提高临床医生对MAS的认识并规范MAS的临床诊断和治疗实践，中华医学会风湿病学分会组织国内专家制定了诊断和治疗标准，以提高MAS的诊治水平并改善患者预后。
