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Early Risk Factors and Clinical Characteristics of Pediatric Hemophagocytic Lymphohistiocytosis: A Single-Center
Ziming Wang1, Zhining Geng2, Yongli Xuan3
1Department of Hematology and Oncology, Children's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, People's Republic of China.
Insights
Early identification of pediatric hemophagocytic lymphohistiocytosis (HLH) is crucial. Fever duration, serum ferritin, triglycerides, fibrinogen, and D-dimer are potential early risk factors for HLH in children.
Area of Science:
- Pediatric Hematology
- Pediatric Immunology
- Critical Care Medicine
Background:
- Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome in children.
- Rapid disease progression and high mortality rates necessitate early diagnosis and intervention.
Purpose of the Study:
- To identify early clinical characteristics and risk factors for pediatric HLH.
- To develop a model for early risk assessment in children with suspected HLH.
Main Methods:
- Retrospective collection of clinical data from 205 children with suspected HLH.
- Application of LASSO-logistic regression to identify significant clinical variables.
- Evaluation of the model's performance using AUC, calibration curves, and bootstrap validation.
Main Results:
- 56 out of 205 patients (27.3%) were diagnosed with HLH.
- Key early risk factors identified include fever duration, serum ferritin (SF), triglycerides (TG), fibrinogen (FIB), and D-dimer (D-D).
- The developed model demonstrated good discriminative ability (AUC = 0.748) and calibration.
Conclusions:
- Fever duration, SF, TG, FIB, and D-D are potential early risk indicators for pediatric HLH.
- These factors can aid in the early risk assessment of children with suspected HLH.
- Timely identification of these factors can facilitate prompt diagnosis and management.
Background:
Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome in children with rapid disease progression and high mortality. Early identification of clinical characteristics and risk-related indicators is essential for timely diagnosis and intervention.
Objective:
To investigate early clinical characteristics and potential early risk-related factors associated with pediatric HLH among children with suspected HLH.
Methods:
We retrospectively collected clinical data from 205 children with suspected HLH who met the inclusion criteria at the Xinjiang Uygur Autonomous Region Children's Hospital between July 2021 and February 2026. Univariate logistic regression, variance inflation factor (VIF) assessment, and LASSO-logistic regression were used to examine clinical variables associated with HLH and to retain potential early risk-related factors. The performance of the final LASSO-logistic regression model was further evaluated using the area under the receiver operating characteristic curve (AUC), calibration curves, bootstrap resampling for internal validation, and the Hosmer-Lemeshow test.
Results:
Of the 205 patients, 56 (27.3%) were diagnosed with HLH. LASSO-logistic regression identified fever duration (OR = 1.551, 95% CI: 0.973-2.472), serum ferritin (SF) (OR = 1.019, 95% CI: 1.001-1.039), triglycerides (TG) (OR = 1.424, 95% CI: 1.003-2.022), fibrinogen (FIB) (OR = 0.832, 95% CI: 0.663-1.044), and D-dimer (D-D) (OR = 1.014, 95% CI: 0.990-1.038) as potential early risk-related factors associated with HLH. The combined model showed the highest discriminative ability, with an AUC of 0.748. The Hosmer-Lemeshow test indicated good calibration (P = 0.721), and bootstrap internal validation yielded a C-index of 0.722.
Conclusion:
Fever duration, SF, D-D, FIB, and TG were potential early risk-related factors associated with pediatric HLH and may provide clinically useful information for early risk assessment in children with suspected HLH.
