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Published on: September 7, 2022
[Clinical Characteristics of Children with Hemophagocytic Syndrome with Different EB Virus DNA Loads]
Jiao-Jiao Huang1, Nan-Du Luo1, Zuo-Chen DU1
1Department of Pediatrics, The Affiliated Hospital of Zunyi Medical University, Guizhou Children's Hospital； Collaborative Innovation Center for Tissue Injury Repair and Regenerative Medicine of Zunyi Medical University， Zunyi 563000， Guizhou Province, China.
Insights
Children with high Epstein-Barr virus (EBV) DNA loads in hemophagocytic lymphohistiocytosis (HLH) show more adverse prognostic factors. Early blood purification therapy is crucial for these high-risk HLH patients, despite similar survival rates across groups.
Area of Science:
- Pediatric Hematology and Oncology
- Infectious Diseases
- Immunology
Context:
- Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome.
- Epstein-Barr virus (EBV) is a common trigger for secondary HLH in children.
- Understanding the impact of EBV viral load on HLH clinical characteristics and prognosis is critical for effective management.
Purpose:
- To analyze clinical features of pediatric HLH based on varying EBV DNA loads.
- To identify differential indicators associated with prognosis in EBV-related HLH.
- To explore the relationship between EBV DNA levels and patient outcomes.
Summary:
- This study analyzed 73 children with HLH, categorizing them by EBV DNA load (negative, low, high).
- Higher EBV DNA loads correlated with increased liver and spleen swelling, central nervous system involvement, and disseminated intravascular coagulation (DIC).
- Low platelet counts and elevated LDH, SF, GGT, and TBIL levels were associated with higher EBV loads. Multivariate analysis identified low platelet counts and CNS dysfunction as independent risk factors for poor prognosis. Despite these differences, survival rates did not significantly vary among groups after treatment, emphasizing the importance of early intervention.
Impact:
- Identifies specific clinical and laboratory markers associated with high EBV viral loads in pediatric HLH.
- Highlights the prognostic significance of low platelet counts and central nervous system dysfunction.
- Underscores the critical role of early blood purification therapy in managing high-risk pediatric HLH patients with high EBV DNA loads.
Objective:
To analyze the clinical characteristics of hemophagocytic syndrome (HLH) children with different EB virus (EBV) DNA loads, and to explore the relationship between differential indicators and prognosis.
Methods:
Clinical data of 73 children with HLH treated in our hospital from January 2015 to April 2022 were collected. According to EBV DNA loads, the children were divided into negative group (≤5×102 copies/ml), low load group (>5×102-<5×105 copies/ml) and high load group (≥5×105copies/ml）. The clinical symptoms and laboratory indexes of the three groups were compared, and the ROC curve was used to determine the best cut-off value of the different indexes. Cox regression model was used to analyze the independent risk factors affecting the prognosis of children, and to analyze the survival of children in each group.
Results:
The proportion of female children, the swelling rate of liver and spleen lymph nodes and the involvement rate of blood, liver, circulation and central nervous system in the high load group were higher than those in the negative group. The incidence of disseminated intravascular coagulation(DIC) and central nervous system(CNS) involvement in the high load group were higher than those in the low load group. The liver swelling rate and circulatory system involvement rate in the low load group were higher than those in the negative group(P<0.05). PLT counts in the high load group were significantly lower than those in the negative group, and the levels of GGT, TBIL, CK-MB, LDH, TG, SF, and organ involvement were significantly higher than those in the negative group. The levels of CK, LDH, SF and the number of organ involvement in the high load group were significantly higher than those in the low load group. The levels of GGT and TBIL in low load group were significantly higher than those in negative group. In terms of treatment, the proportion of blood purification therapy in the high and low load group was significantly higher than that in the negative group(P<0.01). ROC curve analysis showed that the best cut-off values of PLT, LDH, TG and SF were 49.5, 1139, 3.12 and 1812, respectively. The appellate laboratory indicators were dichotomized according to the cut-off value, and the differential clinical symptoms were included in the Cox regression model. Univariate analysis showed that LDH>1139 U/L, SF>1812 μg/L, dysfunction of central nervous system, number of organ damage, DIC and no blood purification therapy were the risk factors affecting the prognosis of children (P<0.05); Multivariate analysis shows that PLT≤49.5×109/L and dysfunction of central nervous system were risk factors affecting the prognosis of children (P<0.05). Survival analysis showed that there was no significant difference in the survival rate among the three groups.
Conclusion:
The incidence of adverse prognostic factors in children with HLH in the EBV-DNA high load group is higher, and there is no significant difference in the survival rate of the three groups after blood purification therapy. Therefore, early identification and application of blood purification therapy is of great significance for children with HLH in the high load group.
