Yin Qingxia, Wang Jingshi, Wang Zhao
Department of Hematology, Beijing Friendship Hospital, Capital Medical University, YongAn Road 95th Xicheng District, Beijing, China.
Ann Hematol. 2022 Nov;101(11):2471-2476. doi: 10.1007/s00277-022-04957-x. Epub 2022 Sep 7.
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a common subtype of secondary HLH. EBV plays an important part in the course. EBV can cause central nervous system (CNS) infections, and there are few clinical studies on EBV-CNS infection in EBV-HLH patients. All patients who were diagnosed as EBV-HLH and underwent cerebrospinal fluid testing admitted to our center from January 2018 to December 2019 were retrospectively analyzed. Summarized the clinical data, evaluated treatment efficacy after intrathecal injection, and investigated the correlation between EBV-CNS infection with prognosis in EBV-HLH patients. Of 37 of 57 (64.9%) EBV-HLH patients has EBV-CNS infection. The survival of EBV-HLH patients without EBV-CNS infection was significantly better than that in EBV-CNS infection patients (P = 0.018). There were no statistically significant differences in sCD25, ferritin, ALT, AST, LDH, TB, WBC, Hb, and PLT counts between two groups (all P-values > 0.05). Higher EBV-DNA load in peripheral blood was correlated with EBV-CNS infection (P < 0.001). EBV-CNS infection is an independent risk factor affecting the survival of patients (P = 0.004). The CSF cell load of patients with and without EBV-CNS infection groups was significantly different (P = 0.024). Intrathecal injection with methotrexate combined with dexamethasone can effectively decrease CSF EBV-DNA load (P = 0.017) and CSF cell load (P = 0.025). EBV-CNS infection is an independent risk factor affecting prognosis in EBV-HLH patients. Therefore, EBV-CNS infection should cause concern for EBV-HLH patients. Cerebrospinal fluid testing is necessary for all patients. Methotrexate combined with dexamethasone intrathecal injection can be an effective treatment for EBV-CNS infection.
爱泼斯坦-巴尔病毒相关噬血细胞性淋巴组织细胞增生症（EBV-HLH）是继发性HLH的常见亚型。EBV在病程中起重要作用。EBV可引起中枢神经系统（CNS）感染，而关于EBV-HLH患者EBV-CNS感染的临床研究较少。对2018年1月至2019年12月在本中心确诊为EBV-HLH并接受脑脊液检测的所有患者进行回顾性分析。总结临床资料，评估鞘内注射后的治疗效果，并探讨EBV-HLH患者EBV-CNS感染与预后的相关性。57例EBV-HLH患者中有37例（64.9%）发生EBV-CNS感染。无EBV-CNS感染的EBV-HLH患者生存率明显高于EBV-CNS感染患者（P = 0.018）。两组间sCD25、铁蛋白、ALT、AST、LDH、TB、WBC、Hb和PLT计数无统计学差异（所有P值>0.05）。外周血中较高的EBV-DNA载量与EBV-CNS感染相关（P < 0.001）。EBV-CNS感染是影响患者生存的独立危险因素（P = 0.004）。有和无EBV-CNS感染组患者的脑脊液细胞载量有显著差异（P = 0.024）。鞘内注射甲氨蝶呤联合地塞米松可有效降低脑脊液EBV-DNA载量（P = 0.017）和脑脊液细胞载量（P = 0.025）。EBV-CNS感染是影响EBV-HLH患者预后的独立危险因素。因此，EBV-CNS感染应引起EBV-HLH患者的关注。所有患者均需进行脑脊液检测。甲氨蝶呤联合地塞米松鞘内注射可有效治疗EBV-CNS感染。
