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<p>Pediatric hemophagocytic lymphohistiocytosis (HLH) lacks reliable risk models for personalized treatment. We aimed to develop a survival nomogram to address this gap. We retrospectively analyzed 404 pediatric HLH patients from multiple centers. Independent prognostic variables identified by Cox regression were incorporated into a nomogram. Five predictors of mortality were identified: central nervous system involvement, gastrointestinal bleeding, WBC &lt; 2.3 × 10⁹/L, Hb &lt; 80&#xa0;g/L, and IL-10 &gt; 69pg/mL. The nomogram demonstrated strong predictive accuracy, with validation cohort AUCs of 0.83, 0.78, and 0.79 for 1-, 2-, and 3-year overall survival (OS). Calibration and decision curve analysis confirmed accuracy. Using an X-tile-determined cutoff of 261, patients were stratified into low- and high-risk groups with significantly different 3-year OS (training cohort: 94.0% vs. 47.2%; validation cohort: 88.2% vs. 43.2%; <i>p</i> &lt; 0.0001). This multicenter study established and externally validated a nomogram-based model that enables early risk stratification and may inform individualized therapy for pediatric HLH.&#xa0;</p>

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Prognostic scoring model incorporating clinical characteristics for pediatric hemophagocytic lymphohistiocytosis: a multicenter retrospective study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年10月16日

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  • Shu-yi Guo,
  • Ru Tang,
  • Shi-lin Liu,
  • Li-hua Yang,
  • Yun-yan He,
  • Liu-hua Liao,
  • Hong Wen,
  • Sen-lin Luo,
  • Xiang Lan,
  • Ya-wei Zou,
  • Hui-qin Chen,
  • Xing-jiang Long,
  • Qi-wen Chen,
  • Hai-xia Guo,
  • Cong Liang,
  • Hao-yan Ren,
  • Si-xi Liu,
  • Dun-hua Zhou

摘要

Pediatric hemophagocytic lymphohistiocytosis (HLH) lacks reliable risk models for personalized treatment. We aimed to develop a survival nomogram to address this gap. We retrospectively analyzed 404 pediatric HLH patients from multiple centers. Independent prognostic variables identified by Cox regression were incorporated into a nomogram. Five predictors of mortality were identified: central nervous system involvement, gastrointestinal bleeding, WBC < 2.3 × 10⁹/L, Hb < 80 g/L, and IL-10 > 69pg/mL. The nomogram demonstrated strong predictive accuracy, with validation cohort AUCs of 0.83, 0.78, and 0.79 for 1-, 2-, and 3-year overall survival (OS). Calibration and decision curve analysis confirmed accuracy. Using an X-tile-determined cutoff of 261, patients were stratified into low- and high-risk groups with significantly different 3-year OS (training cohort: 94.0% vs. 47.2%; validation cohort: 88.2% vs. 43.2%; p < 0.0001). This multicenter study established and externally validated a nomogram-based model that enables early risk stratification and may inform individualized therapy for pediatric HLH.