Clinical warning of hemophagocytic syndrome caused by Epstein-Barr virus

Abstract

Objectives: This study aimed to compare the clinical features and laboratory tests of infectious mononucleosis (IM) and hemophagocytic syndrome (HLH) caused by Epstein-Barr virus (EBV) in 1-3-year-old children and to explore the risk factor of HLH caused by EBV (EBV-HLH).Methods: The clinical data of 92 children with EBV infection admitted in our hospital from 2011 to 2019 were collected; 61 cases were diagnosed as EBV-IM, and 31 cases were diagnosed as EBV-HLH. The subjects’ clinical manifestations and laboratory tests were analyzed retrospectively.Results: Compared with EBV-IM patients, EBV-HLH patients had longer durations of fever, both before hospitalization and overall, and a higher probability of hepatomegaly. The levels of ALT, AST, LDH, TG, SF, D-Dimer and the plasma EBV DNA load of EBV-HLH patients were significantly higher than those of EBV-IM patients. The absolute values of CD3+, CD4+, CD8+, NK, and CD3-CD19+ cells and IgA and IgM levels of EBV-HLH patients were significantly lower than those of EBV-IM patients. The plasma EBV DNA load was positively correlated with the PT, TT, α-HBDH, AST, LDH, CK, Scr, BUN, UA, TG, and CRP levels in EBV-HLH patients, and the plasma EBV DNA load was positively correlated with the D-Dimer level in the EBV-IM patients. Among the 10 different potential markers, at the cut-off point of 1721.500 µg/L, the sensitivity and specificity of D-Dimer was 88.90% and 90.20%, respectively.Conclusion: The D-Dimer level may be a good prognostic indicator of EBV-HLH caused by EBV.

Oncogenicity of EBV-infected natural killer T cells deteriorate EBV-related hemophagocytic lymphohistiocytosis

“…Although HLH was partly de ned by lower NK functionality, the number of NK cells has also been reported to be signi cantly decreased in patients with HLH [12]. However, no threshold was de ned for not only the absolute number but also the functionality of NK cells.…”

“…The high morbidity and mortality of EBV-associated lymphoproliferation are mainly attributed to the poorly understood pathogenesis of EBV infection in T or NK cells. The number of T and NK cells was signi cantly decreased in patients with EBV-HLH, indicating that immune de ciency occurs in EBV-HLH [11,12]. The low frequency of EBV-speci c CD8 + T cells may also be one of the immunological features of CAEBV infection [13].…”