Qiu Kun-Yin, Guo Shu-Yi, Zeng Yang-Hui, Liao Xiong-Yu, Lin Shao-Fen, Fang Jian-Pei, Zhou Dun-Hua
Children's Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, People's Republic of China.
Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, People's Republic of China.
Hematology. 2022 Dec;27(1):874-880. doi: 10.1080/16078454.2022.2109328.
Purpose Our aim is to analyze the clinical characteristics and prognostic factors of Epstein-Barr (EB) virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in children. Methods Children with newly diagnosed HLH were retrospectively analyzed. Results Finally, a total of 95 children with HLH were enrolled in this study, including 43 (45.3%) with EBV-HLH and 52 (54.7%) with non-EBV-HLH. Laboratory tests showed that the levels of absolute neutrophil count (ANC) decrease ( = 0.031) and triglycerides (TG) increase ( = 0.036) in the EBV-HLH group were statistically significant compared with those in the non-EBV-HLH group, respectively. We found that the remission rate during induction period in the EBV-HLH group and non-EBV-HLH group was 75.8% and 89.3%, respectively. The correlation analysis showed that the elevated degree of total bilirubin (TBIL) ( = 0.042), triglyceride (TG) ( = 0.009), serum ferritin (SF) ( = 0.008) and interleukin-8 (IL-8) ( = 0.004) were related with the remission rate during induction in the EBV-HLH group. Further univariate analysis showed that the elevated degree of TBIL ( = 0.048) and TG ( = 0.019) were significant risk factors for the remission rate during induction in the EBV-HLH group. In the multivariate analysis, we observed that there was statistical significance for the degree of TG elevation ( = 0.015) between the two groups. The correlation analysis showed that the elevated degree of TBIL ( = 0.030), the elevated degree of SF ( = 0.020) and the elevated degree of interleukin-6 ( = 0.010) were related to the induction mortality of children with EBV-HLH. Conclusion TBIL and TG are valid indicators to assess the efficacy and prognosis of EVBHLH among children in induction period.
目的 我们的目的是分析儿童爱泼斯坦-巴尔（EB）病毒相关噬血细胞性淋巴组织细胞增生症（EBV-HLH）的临床特征和预后因素。方法 对新诊断的HLH患儿进行回顾性分析。结果 最终，本研究共纳入95例HLH患儿，其中43例（45.3%）为EBV-HLH，52例（54.7%）为非EBV-HLH。实验室检查显示，与非EBV-HLH组相比，EBV-HLH组的绝对中性粒细胞计数（ANC）水平降低（P = 0.031）和甘油三酯（TG）水平升高（P = 0.036）具有统计学意义。我们发现，EBV-HLH组和非EBV-HLH组诱导期的缓解率分别为75.8%和89.3%。相关性分析显示，EBV-HLH组总胆红素（TBIL）升高程度（P = 0.042）、甘油三酯（TG）升高程度（P = 0.009）、血清铁蛋白（SF）升高程度（P = 0.008）和白细胞介素-8（IL-8）升高程度（P = 0.004）与诱导期缓解率相关。进一步单因素分析显示，TBIL升高程度（P = 0.048）和TG升高程度（P = 0.019）是EBV-HLH组诱导期缓解率的显著危险因素。多因素分析中，我们观察到两组间TG升高程度有统计学意义（P = 0.015）。相关性分析显示，TBIL升高程度（P = 0.030）、SF升高程度（P = 0.020）和白细胞介素-6升高程度（P = 0.010）与EBV-HLH患儿的诱导期死亡率相关。结论 TBIL和TG是评估儿童EBV-HLH诱导期疗效和预后的有效指标。
