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.
题目: 不同EB病毒DNA载量的噬血细胞综合征患儿临床特征分析.
目的: 分析不同EB病毒（EBV）DNA载量的噬血细胞综合征（HLH）患儿临床特征差异，探讨差异指标与患儿预后的关系.
方法: 收集2015年1月至2022年4月在我院诊治的共73例HLH患儿的临床资料，将患儿按照EBV拷贝数分为阴性组（≤5×102 copies/ml）、低载量组（>5×102~<5×105 copies/ml）和高载量组（≥5×105 copies/ml）。比较3组患儿的临床症状和实验室指标，采用ROC曲线确定差异指标的最佳截断值。应用Cox回归模型分析影响患儿预后的独立危险因素，并分析各组患儿生存情况.
结果: 高载量组女性患儿比例、肝脾淋巴结肿大率和血液、肝脏、循环、中枢神经系统受累率均高于阴性组；高载量组DIC发生率和中枢神经系统受累率高于低载量组；低载量组肝肿大率及循环系统受累率高于阴性组（P＜0.05）。高载量组PLT计数明显低于阴性组，而GGT、TBIL、CK-MB、LDH、TG、SF水平及器官受累数明显高于阴性组；高载量组CK、LDH、SF水平和器官受累数明显高于低载量组；低载量组GGT和TBIL水平明显高于阴性组（P＜0.01）。在治疗方面，高、低载量组使用血液净化疗法的比例明显高于阴性组患儿。应用ROC曲线分析显示，PLT、LDH、TG及SF的最佳截断值分别为49.5、1139、3.12、1812；将上诉实验室指标按照截断值进行二分类同时将差异临床症状纳入Cox回归模型，单因素分析结果显示，LDH>1139 U/L、SF>1812 μg/L、中枢神经系统功能障碍、器官损害数、DIC及未采用血液净化疗法是影响患儿预后的危险因素（P＜0.05）；多因素分析显示，PLT≤49.5×109/L、中枢神经系统功能障碍是影响患儿预后的危险因素（P＜0.05）。生存分析显示三组患儿生存率无统计学差异.
结论: EBV-DNA高载量组HLH患儿预后不良因素的发生率更高，而经血液净化疗法治疗后三组患儿生存率无统计学差异，因此早期识别并应用血液净化疗法对高载量组HLH患儿具有重要意义.
Keywords: EB virus DNA loads; blood purification; children; hemophagocytic syndrome; prognostic factors.