Huang Jiao-Jiao, Luo Nan-Du, DU Zuo-Chen, Yan Jia-Hong, Ma Jin-Hua, Cao Xiu-Li, He Zhi-Xu, Huang Pei, Chen Yan
Department 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.
Department 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,E-mail：
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2023 Feb;31(1):261-267. doi: 10.19746/j.cnki.issn.1009-2137.2023.01.041.
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.
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 (≤5×10 copies/ml), low load group (>5×10-<5×10 copies/ml) and high load group (≥5×10copies/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.
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(<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(<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 (<0.05); Multivariate analysis shows that PLT≤49.5×10/L and dysfunction of central nervous system were risk factors affecting the prognosis of children (<0.05). Survival analysis showed that there was no significant difference in the survival rate among the three groups.
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.
分析不同EB病毒（EBV）DNA载量的噬血细胞综合征（HLH）患儿的临床特征，探讨差异指标与预后的关系。
收集2015年1月至2022年4月在我院治疗的73例HLH患儿的临床资料。根据EBV DNA载量，将患儿分为阴性组（≤5×10拷贝/ml）、低载量组（>5×10-<5×10拷贝/ml）和高载量组（≥5×10拷贝/ml）。比较三组的临床症状和实验室指标，采用ROC曲线确定不同指标的最佳截断值。采用Cox回归模型分析影响患儿预后的独立危险因素，并分析各组患儿的生存情况。
高载量组女童比例、肝脾淋巴结肿大率及血液、肝脏、循环系统和中枢神经系统受累率均高于阴性组。高载量组弥散性血管内凝血（DIC）和中枢神经系统（CNS）受累发生率高于低载量组。低载量组肝脏肿大率和循环系统受累率高于阴性组（<0.05）。高载量组血小板计数明显低于阴性组，谷氨酰转肽酶（GGT）、总胆红素（TBIL）、肌酸激酶同工酶（CK-MB）、乳酸脱氢酶（LDH）、甘油三酯（TG）、铁蛋白（SF）水平及器官受累情况均明显高于阴性组。高载量组CK、LDH、SF水平及器官受累数量明显高于低载量组。低载量组GGT和TBIL水平明显高于阴性组。治疗方面，高、低载量组血液净化治疗比例明显高于阴性组（<~0.01）。ROC曲线分析显示，血小板、LDH、TG和SF的最佳截断值分别为49.5、1139、3.12和1812。根据截断值将上诉实验室指标进行二分法，并将差异临床症状纳入Cox回归模型。单因素分析显示，LDH>1139 U/L、SF>1812 μg/L、中枢神经系统功能障碍、器官损伤数量、DIC及未进行血液净化治疗是影响患儿预后的危险因素（<0.05）；多因素分析显示，血小板≤49.5×10/L和中枢神经系统功能障碍是影响患儿预后的危险因素（<0.05）。生存分析显示，三组生存率差异无统计学意义。
EBV-DNA高载量组HLH患儿不良预后因素发生率较高，血液净化治疗后三组生存率差异无统计学意义。因此，早期识别并应用血液净化治疗对高载量组HLH患儿具有重要意义。
