This study reviews the clinical features, treatment patterns, and outcomes of pediatric secondary hemophagocytic lymphohistiocytosis (HLH) at a single center. The findings indicate that infections are the most common triggers.
Background
Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that can be life-threatening, particularly in children. Secondary HLH can arise from various conditions, including infections and malignancies.
Data Highlights
Characteristic
Value
Median Age at Diagnosis
34 months
Male Percentage
62.5%
Infection Triggers
56.3%
Complete Remission Rate
81.3%
Overall Survival Rate
87.5%
Key Findings
Infections were the most common trigger for secondary HLH, accounting for 56.3% of cases.
93.8% of patients presented with fever and cytopenias affecting at least two lineages.
All patients exhibited hyperferritinemia at presentation.
Thirteen out of sixteen patients achieved complete remission.
Overall survival was 87.5%, with deaths occurring in high-risk noninfectious cases.
Clinical Implications
Clinicians should be aware of the common triggers and clinical presentations to facilitate timely diagnosis and management.
Conclusion
Pediatric secondary HLH, primarily associated with infections, can have favorable outcomes when diagnosed and treated promptly. However, cases linked to metabolic disorders and LCH may lead to refractory disease and increased mortality.
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