Prognostic scoring model incorporating clinical characteristics for pediatric hemophagocytic lymphohistiocytosis: a multicenter retrospective study
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.
Authors
- Yawei Zou
- Cong Liang (ORCID: https://orcid.org/0000-0002-6796-8417)
- Yunyan He (ORCID: https://orcid.org/0000-0001-7181-4878)
- Hong Wen
- Qi-wen Chen
- Sixi Liu
- Liuhua Liao
- Shu-yi Guo
- Lan Xiang (ORCID: https://orcid.org/0000-0001-6595-2836)
- Haixia Guo (ORCID: https://orcid.org/0009-0008-7187-6209)
- Lihua Yang (ORCID: https://orcid.org/0000-0002-6293-4816)
- Senlin Luo (ORCID: https://orcid.org/0000-0002-3310-8312)
- Shilin Liu (ORCID: https://orcid.org/0000-0001-6854-845X)
- Huiqin Chen (ORCID: https://orcid.org/0000-0003-2477-5436)
- Tang Ru (ORCID: https://orcid.org/0000-0003-3585-8675)
- Dun‐hua Zhou (ORCID: https://orcid.org/0000-0002-8173-4924)
- Han Ren (ORCID: https://orcid.org/0009-0007-8449-8537)
- Xing‐Jiang Long
Institutions
- Central South University (CN)
- Nanchang University (CN)
- Sun Yat-sen University (CN)
- Guangxi Medical University (CN)
- Guangdong Medical College (CN)
- First Affiliated Hospital of Xiamen University (CN)
- Liuzhou General Hospital (CN)
- Sun Yat-sen Memorial Hospital (CN)
- First Affiliated Hospital of Guangzhou Medical University (CN)
- Zhujiang Hospital (CN)
- First Affiliated Hospital of GuangXi Medical University (CN)
- The First Affiliated Hospital, Sun Yat-sen University (CN)
- Weihai Maternal and Child Health Hospital (CN)
- Huizhou Central People's Hospital (CN)
- Third Affiliated Hospital of Sun Yat-sen University (CN)
- Shenzhen Children's Hospital (CN)
- Second Xiangya Hospital of Central South University (CN)
- First Affiliated Hospital of Nanchang University (CN)
- Southern Medical University (CN)
- Guangzhou Medical University (CN)
Publication Details
- Journal
- Annals of Hematology
- Published
- 2025-10-16
- DOI
- https://doi.org/10.1007/s00277-025-06676-5
- Primary Topic
- Autoimmune and Inflammatory Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Sun Yat-sen University
- Guangdong Medical Research Foundation
- Guangzhou First People's Hospital
- Basic and Applied Basic Research Foundation of Guangdong Province