Chunfan Li,* Kebing Lv,* Yulan Zhou, Xiaoye Cheng, Fei Li

Jiangxi Provincial Key Laboratory of Hematological Diseases, Department of Hematology, The First Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, Jiangxi, 330006, People’s Republic of China

*These authors contributed equally to this work

Correspondence: Fei Li, Email ndyfy01238@ncu.edu.cn

Introduction: Hemophagocytic lymphohistiocytosis (HLH), a life-threatening hyperinflammatory syndrome, is characterised by rapid disease progression and high early mortality. We aim to investigate the characteristics of the early death (ED) in adults with HLH and develop a reliable risk model to predict early mortality.
Materials and Methods: Between September 2013 and July 2024, 254 adult patients with HLH were examined. Clinical data including presentation, triggers, treatments, and laboratory results were reviewed. Cases were randomly divided into training and validation cohorts using R. Independent risk factors for 60-day mortality were identified, leading to the development of a risk model using nomogram.
Results: 26.4% (67/254) of patients with HLH died within 60 days. Cox multivariate analyses identified independent risk factors for 60-day mortality, including PLT < 100× 109/L (P = 0.006), sCD25 ≥ 12000 U/mL (P = 0.046) and EBV DNA copies ≥ 10000 copies/mL (P = 0.006). And the C-index, a measure of predictive performance, of the ED risk model by nomogram yielded values of 0.698 and 0.654 in the training cohort and validation cohort, respectively. The calibration curve demonstrated that the prediction outcome was correlated with the observed outcome. Assigning values to each risk factor, this resulted in the stratification of the 254 patients into low-risk groups (n = 54), intermediate-risk groups (n = 184), and high-risk groups (n = 16), with the corresponding 60-day overall survival rates being 90.7%, 71.7%, and 37.5%, respectively.
Conclusion: The novel ED risk model can effectively and precisely identify high-risk adult patients with HLH, offering appropriate clinical recommendations.

Keywords: hemophagocytic lymphohistiocytosis, early mortality, risk model, sCD25, nomogram