Clinical Profile and Outcomes of Pediatric Secondary HLH: Insights from a Tertiary Care Hospital

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory condition seen in children with various etiology. Sepsis and rheumatological etiolgy are being common. This was a retrospective study conducted at a tertiary care hospital during the study period January 2020 to July 2024. Children aged ≤ 14 years, who were diagnosed as HLH/MAS during the period enrolled in the study consecutively. HLH was classified based on the HLH-2004 criteria, except in cases involving underlying rheumatologically conditions, where the 2016 MAS (Macrophage Activation Syndrome) criteria were applied. Data was collected in a predesigned proforma. A total of 26 children were treated for HLH/MAS. The mean age at presentation was 8.8 years (± 3.9), with an equal distribution between males and females. Rheumatologic disorders, primarily systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (SLE), accounted for the majority of cases (58%), followed by infections (34%) and malignancies (8%). In terms of outcomes, 17 patients (65%) survived, while 9 (35%) succumbed to the illness. ferritin and fibrinogen levels showed statistically significant differences between survivor and non-survivor. econdary HLH/MAS is a dreaded complication in children and ferritin remains as an important biochemical marker in predicting outcome.