Epidemiological investigation of hemophagocytic lymphohistiocytosis in China

Orphanet Journal of Rare Diseases

Yan Chen¹  Fan Zhou²  Jie Yu³  Rui Zhang⁴  Shuyan Yao⁵  Zhao Wang⁵  Yini Wang⁵  Yuan Sun⁶  Li Liu⁷  Qiaohua Zhang⁸  Jianpei Fang⁹  Huo Tan^1,10  Yun Zeng^1,11  Fei Li^1,12  Cheng Zhang^1,13  Jie Bai^1,14  Runming Jin^1,15 

[1] Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou, People’s Republic of China;General Hospital of Shenyang Military Region, Shenyang, People’s Republic of China;Hematology and Oncology, Children’s Hospital of Chongqing Medical University, Chongqing, People’s Republic of China;Hematology, Beijing Children’s Hospital, Beijing, People’s Republic of China;Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, People’s Republic of China;Hematology, Beijing Jingdu Children’s Hospital, Beijing, People’s Republic of China;Hematology, Tangdu Hospital, Fourth Military Medical University, Xi’an, People’s Republic of China;Lymphatic Oncology, Shanxi Bethune Hospital, Shanxi, People’s Republic of China;Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, People’s Republic of China;The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, People’s Republic of China;The First Affiliated Hospital of Kunming Medical College, Yunnan, People’s Republic of China;The First Affiliated Hospital of Nanchang University, Jiangxi, People’s Republic of China;The Second Affiliated Hospital of Third Military Medical University, Chongqing, People’s Republic of China;The Second Hospital of Tianjin Medical University, Tianjin, People’s Republic of China;Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, People’s Republic of China;

关键词: Hemophagocytic lymphohistiocytosis;    Epidemiology;    Incidence;    Diagnosis rate;    Gross domestic product;   

DOI  :  10.1186/s13023-021-01976-1

来源: Springer

【 摘 要 】

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.

【 授权许可】

CC BY