Objective: To compare the clinical characteristics of children with hemophagocytic lymphocytosis (HLH) associated with primary Epstein-Barr virus (EBV) infection and EBV reactivation, and explore the effects of different EBV infection status on the clinical indexes and prognosis of HLH.
Methods: The clinical data of 51 children with EBV associated HLH treated in Henan Children's Hospital from June 2016 to June 2021 were collected. According to the detection results of plasma EBV antibody spectrum, they were divided into EBV primary infection-associated HLH group (18 cases) and EBV reactivation-associated HLH group (33 cases). The clinical features, laboratory indexes and prognosis of the two groups were analyzed and compared.
Results: There were no significant differences in age, gender, hepatomegaly, splenomegaly, lymphadenopathy, neutrophil count in peripheral blood, hemoglobin content, platelet count, plasma EBV-DNA load, lactate dehydrogenase, alanine aminotransferase, aspartate aminotransferase, albumin, fibrinogen, triglyceride, ferritin, hemophagocytosis in bone marrow, NK cell activity and sCD25 between the two groups(P>0.05). The central nervous system involvement and CD4/CD8 in EBV reactivation-associated HLH group were significantly higher than those in primary infection-associated HLH group, but the total bilirubin was significantly lower than that in primary infection-associated HLH group (P<0.05). After treatment according to HLH-2004 protocol, the remission rate, 5-year OS rate and 5-year EFS rate of patients in EBV reactivation-associated HLH group were significantly lower than those in EBV primary infection-associated HLH group (P<0.05).
Conclusion: EBV reactivation-associated HLH is more likely to cause central nervous system involvement and the prognosis is worser than EBV primary infection-associated HLH, which requires intensive treatment.
题目: 原发与再激活EB病毒感染相关噬血细胞性淋巴组织细胞增生症的比较分析.
目的: 比较原发性EB病毒（EBV）感染及EBV再激活相关噬血细胞性淋巴组织细胞增生症（HLH）患儿的临床特征，探讨不同EBV感染状态对HLH临床指标及预后的影响.
方法: 收集河南省儿童医院2016年6月至2021年6月收治的51例EBV相关HLH患儿的临床资料，根据血浆EBV抗体谱检测结果，将患者分为EBV原发性感染HLH组（18例）和EBV再激活HLH组（33例）。分析比较两组的临床特征、实验室指标及预后.
结果: 两组患者在年龄、性别、肝肿大、脾肿大、淋巴结肿大、外周血中性粒细胞数、血红蛋白含量、血小板数、血浆EBV-DNA载量、乳酸脱氢酶、谷丙转氨酶、谷草转氨酶、白蛋白、纤维蛋白原、甘油三酯、铁蛋白、骨髓噬血现象、NK细胞活性、sCD25等方面均无显著差异（P>0.05），EBV再激活HLH组中枢神经系统受累及CD4/CD8明显高于原发EBV感染HLH组（P<0.05），而总胆红素明显低于原发EBV感染HLH组（P<0.05）；参照HLH-2004方案治疗后，EBV再激活HLH组患者的缓解率、5年OS率、5年EFS率均明显低于EBV原发感染HLH组（P<0.05）.
结论: EBV再激活HLH比原发感染HLH更易引起中枢受累，预后更差，需加强治疗.
Keywords: Epstein-Barr virus; hemophagocytic lymphocytosis; primary infection; reactivation.