ObjectiveTo describe the etiological spectrum, treatment approaches, clinical and laboratory characteristics in pediatric secondary hemophagocytic lymphohistiocytosis (sHLH) to improve awareness of this severe illness and summarize evolving management strategies.MethodsA retrospective analysis was conducted on 110 children initially diagnosed with sHLH at our hospital between January 1, 2018, and June 30, 2025.ResultsAmong 110 sHLH patients, the median age at diagnosis was 2.67 years (1.17, 5.96), and 52.7% were under 3 years old. Infection-associated HLH accounted for 78.2%, with Epstein-Barr virus (EBV) as the most common trigger (57.3%).
The remaining cases were attributed to rheumatic or malignant diseases. The main clinical manifestations included fever (99.1%), lymphadenopathy (83.6%), splenomegaly (77.3%), and hepatomegaly (66.3%).
Respiratory system involvement was observed in over half of the patients, while central nervous system involvement (CNSI) and multiple organ dysfunction syndrome (MODS) occurred in 22.7% and 12.8% of cases, respectively. Characteristic laboratory abnormalities were highly prevalent, including pancytopenia (especially thrombocytopenia), hyperferritinemia, hypofibrinogenemia, and elevated soluble interleukin-2 receptor (sCD25).
Most patients showed varying degrees of hepatic dysfunction, mainly with elevated enzymes (LDH, AST, ALT, HBDH).
Frontiers in Immunology published a clinical update in Infectious Disease on 24 Apr 2026.
The item focuses on Unveiling pediatric secondary hemophagocytic lymphohistiocytosis: a comprehensive analysis of etiology, diagnosis, and treatment.
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