机构：[1]Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, People’s Republic of China.首都医科大学附属北京友谊医院[2]Hematology, Beijing Jingdu Children’s Hospital, Beijing, People’s Republic of China.首都医科大学附属北京儿童医院[3]Hematology, Tangdu Hospital, Fourth Military Medical University, Xi’an, People’s Republic of China.[4]Hematology, Beijing Children’s Hospital, Beijing, People’s Republic of China.首都医科大学附属北京儿童医院[5]Sun Yat‑Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, People’s Republic of China.中山大学附属第二医院[6]Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, People’s Republic of China.华中科技大学同济医学院附属协和医院[7]Hematology and Oncology, Children’s Hospital of Chongqing Medical University, Chongqing, People’s Republic of China.[8]The First Affiliated Hospital of Nanchang University, Jiangxi, People’s Republic of China.[9]The Second Hospital of Tianjin Medical University, Tianjin, People’s Republic of China.[10]The First Affiliated Hospital of Kunming Medical College, Yunnan, People’s Republic of China.昆明医科大学附属第一医院[11]The Second Affiliated Hospital of Third Military Medical University, Chongqing, People’s Republic of China.[12]The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, People’s Republic of China.[13]General Hospital of Shenyang Military Region, Shenyang, People’s Republic of China.[14]Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou, People’s Republic of China.[15]Lymphatic Oncology, Shanxi Bethune Hospital, Shanxi, People’s Republic of China.山西白求恩医院
BackgroundCurrently, most research on hemophagocytic lymphohistiocytosis (HLH) have focused on etiology and therapy, leaving few epidemiological reports. The published studies of China are mainly regional investigations. We aimed to present the overall epidemiological status of HLH in China, and provide Chinese data for the international HLH epidemiological investigation.MethodsThe data of HLH cases in China in 2019 were collected and statistically analyzed.FindingsEpstein-Barr virus accounted for 44.01% of the 1445 cases in 31 regions and was the most common cause. Lymphoma-associated HLH patients were more often male (P<0.05) while rheumatic and immune-associated HLH were more often female (P<0.001). Primary HLH and Epstein-Barr Virus-associated HLH were predominant in children (P<0.001) while tumor-associated HLH was predominant in adults. Lymphoma-associated HLH was positively correlated with the age of onset (P<0.01). The diagnosis rate of 29 areas had a significant correlation with per capita Gross domestic product (P<0.05).ConclusionThe different distribution of HLH etiology by age and gender contributes to the diagnosis of HLH by clinicians; The suboptimal diagnosis rate in regions with a high incidence of HLH in China is a result of the effect of the local economic level indicating the importance of improving the regional medical level.
基金：
This work was supported by Beijing Municipal Administration of Hospital
Clinical Medicine Development of Special Funding Support (XMLX201823)
in collection of data and writing the manuscript; National Natural Science
Foundation of China (81871633) in analysis, and interpretation of data; Beijing
Natural Science Foundation (7181003) in analysis, and interpretation of data;
Beijing Municipal Administration of Hospitals’ Ascent Plan (DFL20180101) in
the design of the study.
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外文
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PubmedID：
中科院(CAS)分区：
出版当年[2021]版：
大类|3 区医学
小类|3 区遗传学3 区医学：研究与实验
最新[2025]版：
大类|2 区医学
小类|2 区遗传学2 区医学：研究与实验
JCR分区：
出版当年[2020]版：
Q2GENETICS & HEREDITYQ2MEDICINE, RESEARCH & EXPERIMENTAL
最新[2023]版：
Q2GENETICS & HEREDITYQ2MEDICINE, RESEARCH & EXPERIMENTAL
第一作者机构：[1]Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, People’s Republic of China.
共同第一作者：
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推荐引用方式(GB/T 7714)：
Shuyan Yao,Yini Wang,Yuan Sun,et al.Epidemiological investigation of hemophagocytic lymphohistiocytosis in China[J].ORPHANET JOURNAL OF RARE DISEASES.2021,16(1):342.doi:10.1186/s13023-021-01976-1.
APA：
Shuyan Yao,Yini Wang,Yuan Sun,Li Liu,Rui Zhang...&Zhao Wang.(2021).Epidemiological investigation of hemophagocytic lymphohistiocytosis in China.ORPHANET JOURNAL OF RARE DISEASES,16,(1)
MLA：
Shuyan Yao,et al."Epidemiological investigation of hemophagocytic lymphohistiocytosis in China".ORPHANET JOURNAL OF RARE DISEASES 16..1(2021):342
