Abstract Objective

To analyze the clinical characteristics, prognosis factors and risk factors of chronic active Epstein-Barr virus (EBV) infection in children.

Study design

Observational analysis of baseline data and follow-up evaluation data of children with chronic active EBV infection in our center between January 1, 2016 and December 31, 2019, and were followed through June 30, 2020.

Results

There were 96 children with chronic active EBV infection, including 50 males and 46 females, with the median age of 6.7 years (range from 0.6-17.6 years) at diagnosis. The median follow-up time was 16.5 months. The three most common clinical manifestations were fever, lymph node enlargement, and hepatomegaly or splenomegaly. Thirty-three patients (36.3%) also had a diagnosis of hemophagocytic lymphohistiocytosis (HLH). EBV infected only T lymphocytes, NK cells, or both T- and NK-cell types in 15 (33.3%), 17 (37.8%), and 13 (28.9%), respectively. At the end of follow up, 26 children had died and 60 survived, 10 were lost to follow up. Generally, progression-free survival was 69.8% ± 2.4%. The level of ‘IL-6 and IL-10’ and the combination of ‘younger age and lower pathologic grade’ at diagnosis were independent prognostic factors by Cox regression analysis (P = .009 and 0.018, respectively).

Conclusions

Children with lower levels of IL-6 and IL-10, or with younger age and lower pathologic grades, generally had favorable outcomes at the terminal point of follow up, indicating better prognostic signs.

Article InfoPublication History

Accepted: July 6, 2021

Received in revised form: July 1, 2021

Received: February 25, 2021

Publication stageIn Press Accepted ManuscriptFootnotes

The authors declare no conflicts of interest.

Data Sharing Statement: Deidentified individual participant data (including data dictionaries) will be made available, in addition to study protocols, the statistical analysis plan, and the informed consent form. The data will be made available upon publication to researchers who provide a methodologically sound proposal for use in achieving the goals of the approved proposal. Proposals should be submitted to [email protected]

Contributors’ Statement:

Dr Sitong Chen, Dr Ang Wei and Dr Honghao Ma conceptualized and designed the study, and drafted the initial manuscript.

Dr Liping Zhang, Dr Hongyuan Lian and Dr Yunze Zhao, designed the data collection instruments, collected data.

Dr Qing Zhang, Dr Fenfen Cheng and Dr Dong Wang carried out the initial analyses, and reviewed and revised the manuscript.

Dr Tianyou Wang, Dr Rui Zhang and Dr Zhigang Li conceptualized and designed the study, coordinated, and supervised data collection, and critically reviewed the manuscript for important intellectual content.

All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Acknowledgements: We thank all the patients and their families for their kind cooperation. This work was supported by the Special Fund of the Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority (XTZD20180202), Beijing Municipal Science & Technology Commission (No. 7214226). National Science and Technology Key Projects (No. 2017ZX09304029001)， Scientific Research Common Program of Beijing Municipal Commission of Education (KM202110025011).

Identification

DOI: https://doi.org/10.1016/j.jpeds.2021.07.009

Copyright

© 2021 Elsevier Inc. All rights reserved.

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