AUTHOR=Cui Tingting , Wang Jingshi , Wang Zhao 

TITLE=The Outcome of Induction Therapy for EBV-Related Hemophagocytic Lymphohistiocytosis: A Model for Risk Stratification

JOURNAL=Frontiers in Immunology

VOLUME=Volume 13 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2022.876415

DOI=10.3389/fimmu.2022.876415

ISSN=1664-3224

ABSTRACT=abnormal inflammation caused by Epstein-Barr virus (EBV) infection, which has high mortality during induction therapy.
Objectives: This study aimed to build a model to predict the risk of the death during induction therapy.
Methods: The EBV-HLH patients admitted from January 2015 to December 2018 were retrospectively reviewed. The primary outcome was death during induction therapy. The interval from receiving therapy to death or the end of induction therapy was the observing time. The patients admitted from Jan 2015 -Dec 2017 were assigned to the primary group, and the patients admitted from Jan to Dec 2018 were set as the validation group.
Results: We included 234 EBV-HLH patients, of whom 65 (27.4%) died during induction therapy. The middle observing time was 25 days. Based on the primary group, the multivariate Cox analysis demonstrated age >18 years, blood urea nitrogen, procalcitonin >2 ug/L, serum CD25 and EBV-DNA in peripheral blood mononuclear cell as the risk factors of death during induction therapy. We developed a nomogram integrating the above factors with high predictive accuracy (c-statistic, 0.86), and stratified all patients into the high-risk and the low-risk group. Based on the validation group, the high-risk patients had a higher risk of death (hazard ratio, 4.93; P=0.012). In the subgroup analysis based on patients receiving etoposide-based strategy, the mortality in high-risk and low-risk patients was 43.9 and 3.1 per 100 person-weeks, respectively.
Conclusion: We developed a nomogram for risk stratification of EBV-HLH patients receiving induction therapy.