Epidemiological investigation of hemophagocytic lymphohistiocytosis in China

Abstract

Background 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. Methods The data of HLH cases in China in 2019 were collected and statistically analyzed. Findings 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). Conclusion 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.

Clinical Features and Prognostic Risk Prediction of Non-Hodgkin Lymphoma-Associated Hemophagocytic Syndrome

“…According to the epidemiological survey of HLH in China, the incidence of HLH in 2019 was 1.04/1,000,000, in which the incidence of LAHS was positively correlated with the age of onset ( 38 ). In this study, we mainly wanted to elucidate the clinical features of LAHS and its correlation with pathological types, and did not address the prevalence of LAHS; therefore, a large number of LAHS cases would be beneficial for our study.…”

Spectrum and trigger identification of hemophagocytic lymphohistiocytosis in adults: A single-center analysis of 555 cases

“…The diagnosis of HLH was established according to the HLH-2004 criteria. Cases of HLH should fulfill 5 or more of following criteria: (1) Body temperature ≥ 38.5°C; (2) Splenomegaly; (3) Cytopenia affecting at least 2 of 3 lineages in the peripheral blood: hemoglobin < 90 g/L, platelets < 100×10 9 /L or neutrophils < 1×10 9 /L; (4) Hypertriglyceridemia and/or hypofibrinogenemia: triacylglycerol ≥ 3 mmol/L, or fibrinogen ≤ 1.5 g/L; (5) Serum ferritin ≥ 500 µg/L; (6) Hemophagocytosis found in BM or spleen or lymph nodes; (7) Soluble CD25 (sCD25) ≥ 2400 U/mL; (8) Natural killer (NK) cell activity is low or absent.…”

“…Currently, most data regarding HLH in adults come from small cohorts. Although one recent large multicenter study including 500 adults with HLH was recently published, a detailed description of the underlying causes was not available (9). For instance, for patients with lymphoma, detailed information about the pathological subtypes was not provided (9).…”

“…Although one recent large multicenter study including 500 adults with HLH was recently published, a detailed description of the underlying causes was not available (9). For instance, for patients with lymphoma, detailed information about the pathological subtypes was not provided (9). In this study, we did a single-center analysis of 555 cases of HLH in adults.…”

Epstein-Barr Virus-Positive Lymphoma-Associated Hemophagocytic Syndrome: A Retrospective, Single-Center Study of 51 Patients

“…An epidemiological investigation of all HLH cases in China was conducted in 2019. In the general Chinese population, the incidence of LAHS was 0.014/100,000 per year ( 10 ). HLH can occur not only upon lymphoma onset, but also during relapse or advanced stage of lymphoma.…”