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<p>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, <i>p</i> &lt; 0.001); splenomegaly (HR 2.112, 95% CI 1.178–3.787, <i>p</i> = 0.012); platelet (PLT) count (HR 0.992, 95% CI 0.984–0.999, <i>p</i> = 0.035); aspartate aminotransferase (AST) (HR 1.002, 95% CI 1.001–1.003, <i>p</i> &lt; 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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月10日

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  • Ziheng Wang,
  • Jiaxu Song,
  • Yuxin Li,
  • Jiayi Zhao,
  • Yifei Ma,
  • Zijie Gan,
  • Yongqiu Di,
  • Ruonan Shao,
  • Zijian Li,
  • Yilin Zhu,
  • Lu Zhang,
  • Qingqi Meng,
  • Hongyu Bao,
  • Liubo Zhang,
  • Suyu Jiang,
  • Xue Han,
  • Wanru Chen,
  • Yijing Zhang,
  • Zhangbiao Long,
  • Jianyong Li,
  • Xiaofeng Shi

摘要

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.