Nomogram for predicting the 30- and 90-day prognoses of hemophagocytic lymphohistiocytosis patients: a multicenter and retrospective study

Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal disease with a low survival rate. It is important to identify the patients at risk of poor prognosis among HLH patients. In this multicenter and retrospective study, we reviewed 90 newly diagnosed HLH patients treated at the Second Affiliated Hospital of Nanjing Medical University and the First Affiliated Hospital of Anhui Medical University from April 2014 to February 2025. Four pre-treatment clinical characteristics of HLH patients were confirmed to be the independent risk factors: age (hazard ratio (HR) 1.041, 95% confidence interval (CI)1.023-1.059, p < 0.001); splenomegaly (HR 2.112, 95% CI 1.178-3.787, p = 0.012); platelet (PLT) count (HR 0.992, 95% CI 0.984-0.999, p = 0.035); aspartate aminotransferase (AST) (HR 1.002, 95% CI 1.001-1.003, p < 0.001). Subsequently, the patients' prognostic risk scores were calculated based on these four risk factors to stratify patients to high-risk and low-risk groups. Besides, these four factors were included in the final clinical prediction model. Receiver operating characteristic (ROC) curves revealed that this model had a good discrimination with optimism-corrected area under the curve (AUC) values of 0.78 (95% CI: 0.68-0.88) for 30-day mortality and 0.82 (95% CI: 0.74-0.92) for 90-day mortality. The calibration curves aligned with the model predictions and actual observations. The decision curve analysis also confirmed our model's robust predictive power. These results point out that age, splenomegaly, PLT, and AST levels are independent indicators of overall survival in patients with HLH and that the proposed model has a good prognostic value.

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Publication Details

Journal
Annals of Hematology
Published
2025-11-10
DOI
https://doi.org/10.1007/s00277-025-06718-y
Citations
2
Primary Topic
Autoimmune and Inflammatory Disorders Research
Type
article
Field-Weighted Citation Impact
3.54

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article

Nomogram for predicting the 30- and 90-day prognoses of hemophagocytic lymphohistiocytosis patients: a multicenter and retrospective study

Jing Zhao, Ruonan Shao, Xiaofeng Shi, Yifei Ma et al.
2 citations
Annals of Hematology
Autoimmune and Inflammatory Disorders Research
3.54
article

Nomogram for predicting the 30- and 90-day prognoses of hemophagocytic lymphohistiocytosis patients: a multicenter and retrospective study

Jing Zhao, Ruonan Shao, Xiaofeng Shi, Yifei Ma, Liubo Zhang, Hongyu Bao, Wanru Chen, Yuxin Li, Lu Zhang, Zijian Li, Jianyong Li, Yijing Zhang, Suyu Jiang, Xue Han, Qingqi Meng, Yan Zhu, Z.G. Gan, Zhangbiao Long, J. Song, Di Yang, Ziheng Wang
article en
2 citations

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal disease with a low survival rate. It is important to identify the patients at risk of poor prognosis among HLH patients. In this multicenter and retrospective study, we reviewed 90 newly diagnosed HLH patients treated at the Second Affiliated Hospital of Nanjing Medical University and the First Affiliated Hospital of Anhui Medical University from April 2014 to February 2025. Four pre-treatment clinical characteristics of HLH patients were confirmed to be the independent risk factors: age (hazard ratio (HR) 1.041, 95% confidence interval (CI)1.023-1.059, p < 0.001); splenomegaly (HR 2.112, 95% CI 1.178-3.787, p = 0.012); platelet (PLT) count (HR 0.992, 95% CI 0.984-0.999, p = 0.035); aspartate aminotransferase (AST) (HR 1.002, 95% CI 1.001-1.003, p < 0.001). Subsequently, the patients' prognostic risk scores were calculated based on these four risk factors to stratify patients to high-risk and low-risk groups. Besides, these four factors were included in the final clinical prediction model. Receiver operating characteristic (ROC) curves revealed that this model had a good discrimination with optimism-corrected area under the curve (AUC) values of 0.78 (95% CI: 0.68-0.88) for 30-day mortality and 0.82 (95% CI: 0.74-0.92) for 90-day mortality. The calibration curves aligned with the model predictions and actual observations. The decision curve analysis also confirmed our model's robust predictive power. These results point out that age, splenomegaly, PLT, and AST levels are independent indicators of overall survival in patients with HLH and that the proposed model has a good prognostic value.

Annals of HematologyVol. 104(11)
Anhui Medical University (CN), First Affiliated Hospital of Anhui Medical University (CN), Second Affiliated Hospital of Nanjing Medical University (CN), Nanjing Medical University (CN)
National Natural Science Foundation of China, Government of Jiangsu Province, National Health Commission of the People's Republic of China
Openalex Percentile: Top 6%
Autoimmune and Inflammatory Disorders Research
3.54
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