The effect of Epstein-Barr virus infection in central nervous system on prognosis of patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

Background This study was conducted to investigate the effect of Epstein–Barr virus (EBV) infection in the central nervous system(CNS) on the prognosis of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) patients. Methods Clinical information of 88 EBV-HLH patients diagnosed and treated in our medical center from January 1, 2017 to September 1, 2019 was included. Results The 88 patients were divided into 2 groups based on whether the result of their CSF EBV-DNA testing was positive. Statistical difference in survival time was found between the EBV + group and EBV- group(44 vs. 44 ; P = 0.049). The 88 patients were separated into 2 groups according to whether underwent allogenic hematopoietic stem cell transplantation (allo-HSCT). There were statistically differences in the allo-HSCT group and non allo-HSCT group (60 vs. 28 ;P = 0.040). Among the 60 EBV-HLH patients who did undergo allo-HSCT, they were divided into 2 groups in the light of whether the result of CSF EBV-DNA testing was positive. Statistical difference in survival time was not found between the EBV + group and EBV - group( 32 vs. 28; P = 0.540). Among the 28 EBV-HLH patients who did not undergo allo-HSCT, they were divided into 2 groups based on whether the result of CSF EBV-DNA testing was positive. Significant statistical difference in survival time was found between the EBV + group and EBV - group(12 vs. 16; P = 0.002). The 44 EBV-HLH patients who were EBV-DNA-positive in their CSF were separated into 2 groups for survival time analysis according to the highest copy number of EBV-DNA in the CSF, using 1.0E + 04 copies/ml as the cutoff, and statistical difference in survival time was found between the two groups(P = 0.030). Conclusions EBV infection of the CNS was found to be a poor prognostic factor in EBV-HLH patients. The changes in EBV-DNA in CSF and the EBV-DNA copy number were found to affect the prognosis of EBV-HLH patients. Monitoring the changes in EBV-DNA copy number in the CSF was of great significance. Allo-HSCT improved the prognosis of EBV-HLH patients, including patients who tested positive for EBV-DNA in the CSF.