PurposeThis retrospective study aimed to investigate the clinical characteristics, management, and prognosis of pediatric malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH).MethodsThis was a retrospective, single-center cohort study, pediatric patients diagnosed with M-HLH diagnosed from January 2003 to December 2024 in our center were enrolled. Clinical characteristics, treatment regimens, overall response rate (ORR), and overall survival (OS) were evaluated.
Univariate and multivariate analyses of potential factors were performed to identify prognostic factors.ResultsOf the 44 patients, the median age at M-HLH diagnosis was 6.63 years (range: 0.33–15.58). Most patients (79.5%) developed HLH induced by tumors.
Lymphoma was the most common malignancy (56.8%), predominantly of the T/NK - cell subtype (36.4%), followed by acute leukemia (27.3%) and Langerhans cell histiocytosis (15.9%). Marked elevations were observed in IL - 2R, IFN - γ, TNF - α, IL - 6, and IL - 10.
Pathogenic variants in HLH - associated genes (LYST, UNC13D, and XIAP) were identified in three cases.
Frontiers in Immunology published a clinical update in Infectious Disease on 07 May 2026.
The item focuses on Childhood malignancy-associated hemophagocytic lymphohistiocytosis: a retrospective, single-center study of 44 patients.
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