Epidemiological investigation of hemophagocytic lymphohistiocytosis in China

Shuyan Yao,Yini Wang,Yuan Sun,Fei Li,Qiaohua Zhang,Li Liu,Jianpei Fang,Zhao Wang,Fan Zhou,Huo Tan,Jie Bai,Yan Chen,Rui Zhang,Cheng Zhang,Jie Yu,Runming Jin,Yun Zeng

doi:10.1186/s13023-021-01976-1

Abstract

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.

Highlights

Most research on hemophagocytic lymphohistiocytosis (HLH) have focused on etiology and therapy, leaving few epidemiological reports
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
Case selection Inclusion criteria: ➀ New HLH cases diagnosed according to HLH-2004, that is, which meet at least 5 of the 8 diagnostic criteria of HLH-2004: (1) Body temperature ≥ 38.5°C; (2) Splenomegaly; (3) Cytopenia affecting at least 2 of 3 lineages in the peripheral blood: hemoglobin (HB) < 90 g/L, platelets (PLT) < 100 × 10^9/L or neutrophils (N) < 1 × 10^9/L; (4) Hypertriglyceridemia and/or hypofibrinogenemia: triacylglycerol (TG) ≥ 3 mmol/L, or fibrinogen (Fbg) ≤ 1.5 g/L; (5) Serum ferritin (SF) ≥ 500 μg/L; (6) Hemophagocytosis found in bone marrow or spleen or lymph nodes; (7) Soluble CD25( sCD25) ≥ 2400U/mL; (8) Natural killer (NK) cell activity is low or absent

Summary

Introduction

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. Hemophagocytic syndrome (HPS), known as hemophagocytic lymphohistiocytosis (HLH), is a clinical syndrome caused by inherited or acquired immune dysfunction [1]. The disease is dangerous, progressing rapidly, and has a high mortality [2]. Most research on HLH has focused on etiology and therapy, leaving few epidemiological reports. Yao et al Orphanet J Rare Dis (2021) 16:342 epidemiological status of HLH in China and provide Chinese data for the international HLH epidemiological investigation

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