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<p><i>Purpose</i>.&#xa0;A previously study proposed a three-step screening procedure for pediatric hemophagocytic lymphohistiocytosis (HLH). This procedure includes an HLH screening model referred to as HLH-screen. This study aims to validate the three-step screening procedure and the HLH-Screen in an independent cohort of children, as well as to optimize the screening process for HLH.&#xa0;<i>Patients and methods</i>.&#xa0;A multicenter retrospective study was conducted on children with fever or splenomegaly hospitalized in three hospitals in Hunan Province. The screening performance of the three-step screening procedure and the simplified procedure using HLH-Screen alone were evaluated. Clinical characteristics of patients misclassified as false negatives or false positives by HLH-Screen were analyzed.&#xa0;<i>Results</i>.&#xa0;Medical records of 5294 children with fever or splenomegaly were collected. The overall sensitivity and specificity of the three-step screening procedure were 91.0–93.7% and 91.5–91.7%, respectively. When directly applying the HLH-Screen to the study population, the sensitivity was 89.7–93.7%, and the specificity was 92.2–92.4%. Four false-negative cases lacked cytopenias, two lacked splenomegaly, and all four experienced less severe organ damage. The most common false positive diagnoses were malignancy complicated with sepsis (25.4%), infectious mononucleosis (23.9%), and malignancy complicated with respiratory infections (7.9%).&#xa0;<i>Conclusion</i>.&#xa0;Both screening procedures showed strong and consistent performance in screening for pediatric HLH. While the sensitivity of the three-step procedure slightly outperformed that of the HLH-Screen alone, using the HLH-Screen directly is more convenient. The HLH-Screen-based HLH screening procedure uses simple parameters to rapidly identify high-risk HLH patients, making it applicable in resource-limited settings.</p>

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Optimizing hemophagocytic lymphohistiocytosis screening in children: validation of the HLH-Screen score

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年6月20日

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  • Xiao Li,
  • Zhexuan Tang,
  • Lifang Zhou,
  • Xun Li,
  • Lihui Deng,
  • Haipeng Yan,
  • Xiangyu Wang,
  • Longlong Xie,
  • Ting Luo,
  • Yufan Yang,
  • Xinping Zhang,
  • Jiaotian Huang,
  • Yefei Lei,
  • Zhenghui Xiao,
  • Xiulan Lu

摘要

Purpose. A previously study proposed a three-step screening procedure for pediatric hemophagocytic lymphohistiocytosis (HLH). This procedure includes an HLH screening model referred to as HLH-screen. This study aims to validate the three-step screening procedure and the HLH-Screen in an independent cohort of children, as well as to optimize the screening process for HLH. Patients and methods. A multicenter retrospective study was conducted on children with fever or splenomegaly hospitalized in three hospitals in Hunan Province. The screening performance of the three-step screening procedure and the simplified procedure using HLH-Screen alone were evaluated. Clinical characteristics of patients misclassified as false negatives or false positives by HLH-Screen were analyzed. Results. Medical records of 5294 children with fever or splenomegaly were collected. The overall sensitivity and specificity of the three-step screening procedure were 91.0–93.7% and 91.5–91.7%, respectively. When directly applying the HLH-Screen to the study population, the sensitivity was 89.7–93.7%, and the specificity was 92.2–92.4%. Four false-negative cases lacked cytopenias, two lacked splenomegaly, and all four experienced less severe organ damage. The most common false positive diagnoses were malignancy complicated with sepsis (25.4%), infectious mononucleosis (23.9%), and malignancy complicated with respiratory infections (7.9%). Conclusion. Both screening procedures showed strong and consistent performance in screening for pediatric HLH. While the sensitivity of the three-step procedure slightly outperformed that of the HLH-Screen alone, using the HLH-Screen directly is more convenient. The HLH-Screen-based HLH screening procedure uses simple parameters to rapidly identify high-risk HLH patients, making it applicable in resource-limited settings.