Objective <p>To analyze the clinical characteristics, laboratory data and treatment plan of hemophagocytic lymphohistiocytosis (HLH).</p> Methods <p>A total of 48 cases of HLH admitted to Hebei Children's Hospital from January 2019 to December 2022 and divided into an EBV-HLH group (n = 34) and non-EBV-HLH group (n = 14) based on whether they had eb virus (EBV) infection associated HLH. Demographic data, clinical characteristics, and laboratory data were analyzed separately in both groups.</p> Results <p>Among the 48 pediatric HLH cases, 34 EBV infections (70.8%) was significantly higher than that of other pathogens. Clinical characteristics and laboratory data in the EBV group showed that the number of cases with lymphadenopathy was significantly higher in the EBV group than in the non-EBV-HLH group (P = 0.021), although there was no significant difference in hepatosplenomegaly between the two groups. Total liver function index bilirubin (TB) was significantly higher in the EBV-HLH group than in the non-EBV-HLH group (P = 0.012). Cytokine testing showed increased interleukin-10 (IL-10) and interferon-IFN-γ) in both groups, and more significantly in the EBV-HLH group, as shown by statistical analysis (P = 0.023). Other laboratory data, such as leukocyte (WBC) count, platelet (PLT) count, alanine transaminase (ALT), aspartate aminotransferase (AST), ferritin (FERR), triglycerides (TG), fibrinogen (FIB), showed no significant differences between the two groups. In terms of treatment, the number of cases using etoposide was higher in the EBV-HLH group than in the non-EBV-HLH group, which was statistically significant (P = 0.001). However, there were no significant differences in the use of intravenous immunoglobulin, blood purification, or ventilators between the two groups. Additionally, there were no significant differences in the number of cases with multiple organ dysfunction syndrome (MODS) or survival rates between the two groups.</p> Conclusion <p>EBV is the most common pathogen associated with infection-related HLH in children. Compared with non-EBV-HLH, pediatric EBV-HLH exhibits distinct clinical and laboratory characteristics, with lymphadenopathy and elevated levels of IL-10, IFN-γ, and Total bilirubin (TB) being more prevalent, warranting attention and differentiation.</p>

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Case–Control Analysis of 48 Pediatric Infection-Associated Hemophagocytic Lymphohistiocytosis

  • Gailing Hao,
  • Weili Guo,
  • Xiao Wang,
  • Pengli Hao,
  • Zhao Pei,
  • Lian Jiang

摘要

Objective

To analyze the clinical characteristics, laboratory data and treatment plan of hemophagocytic lymphohistiocytosis (HLH).

Methods

A total of 48 cases of HLH admitted to Hebei Children's Hospital from January 2019 to December 2022 and divided into an EBV-HLH group (n = 34) and non-EBV-HLH group (n = 14) based on whether they had eb virus (EBV) infection associated HLH. Demographic data, clinical characteristics, and laboratory data were analyzed separately in both groups.

Results

Among the 48 pediatric HLH cases, 34 EBV infections (70.8%) was significantly higher than that of other pathogens. Clinical characteristics and laboratory data in the EBV group showed that the number of cases with lymphadenopathy was significantly higher in the EBV group than in the non-EBV-HLH group (P = 0.021), although there was no significant difference in hepatosplenomegaly between the two groups. Total liver function index bilirubin (TB) was significantly higher in the EBV-HLH group than in the non-EBV-HLH group (P = 0.012). Cytokine testing showed increased interleukin-10 (IL-10) and interferon-IFN-γ) in both groups, and more significantly in the EBV-HLH group, as shown by statistical analysis (P = 0.023). Other laboratory data, such as leukocyte (WBC) count, platelet (PLT) count, alanine transaminase (ALT), aspartate aminotransferase (AST), ferritin (FERR), triglycerides (TG), fibrinogen (FIB), showed no significant differences between the two groups. In terms of treatment, the number of cases using etoposide was higher in the EBV-HLH group than in the non-EBV-HLH group, which was statistically significant (P = 0.001). However, there were no significant differences in the use of intravenous immunoglobulin, blood purification, or ventilators between the two groups. Additionally, there were no significant differences in the number of cases with multiple organ dysfunction syndrome (MODS) or survival rates between the two groups.

Conclusion

EBV is the most common pathogen associated with infection-related HLH in children. Compared with non-EBV-HLH, pediatric EBV-HLH exhibits distinct clinical and laboratory characteristics, with lymphadenopathy and elevated levels of IL-10, IFN-γ, and Total bilirubin (TB) being more prevalent, warranting attention and differentiation.