Title |
Epidemiological investigation of hemophagocytic lymphohistiocytosis in China
|
---|---|
Published in |
Orphanet Journal of Rare Diseases, August 2021
|
DOI | 10.1186/s13023-021-01976-1 |
Pubmed ID | |
Authors |
Shuyan Yao, Yini Wang, Yuan Sun, Li Liu, Rui Zhang, Jianpei Fang, Runming Jin, Jie Yu, Fei Li, Jie Bai, Yun Zeng, Cheng Zhang, Huo Tan, Fan Zhou, Yan Chen, Qiaohua Zhang, Zhao Wang |
Abstract |
Currently, 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. The data of HLH cases in China in 2019 were collected and statistically analyzed. Epstein-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). The 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. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 1 | 100% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 1 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 12 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Other | 2 | 17% |
Unspecified | 1 | 8% |
Researcher | 1 | 8% |
Student > Master | 1 | 8% |
Unknown | 7 | 58% |
Readers by discipline | Count | As % |
---|---|---|
Unspecified | 1 | 8% |
Biochemistry, Genetics and Molecular Biology | 1 | 8% |
Nursing and Health Professions | 1 | 8% |
Unknown | 9 | 75% |