Recommendations for the diagnosis and treatment of rheumatic diseases-related hemophagocytic syndrome in China
10.3760/cma.j.cn112138-20221010-00749
- VernacularTitle:风湿性疾病相关噬血细胞综合征诊疗规范
- Author:
Qian WANG
1
;
Yini WANG
;
Qiang WANG
;
Miaojia ZHANG
;
Hongsheng SUN
;
Chongyang LIU
;
Zhao WANG
;
Yan ZHAO
Author Information
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
- Keywords:
Hemophagocytic syndrome;
Macrophage activation syndrome;
Lymphohistiocytosis,hemophagocytic;
Autoimmune diseases;
Autoinflammatory disorders;
Diagnosis;
Tre
- From:
Chinese Journal of Internal Medicine
2023;62(1):23-30
- CountryChina
- Language:Chinese
-
Abstract:
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.
