Critical care outcomes of hemophagocytic lymphohistiocytosis in children: a single centre retrospective observational study
Authors' conclusion
In this cohort, mortality among children admitted to the PICU with HLH was high, particularly among those with primary or onco-hematological forms. Conversely, patients with MAS experienced more relapses but no mortality was observed in this sample. Early presence of splenomegaly, low platelet count, elevated ferritin levels, and PIM2 score at PICU admission appear to be indicators of poor prognosis.
Study context
- Population
- Children with hemophagocytic lymphohistiocytosis (HLH) admitted to the pediatric intensive care unit (PICU), 2006–2019
- Participants
- 48 children
- Exposure
- Non-survivors vs survivors; pHLH vs sHLH vs macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA)
- pHLHn = 5
- sHLHn = 37
- MASn = 6
- Outcome
- PICU mortality
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
- Overall PICU mortality
- 33.3%
Observational data: associations, not effects.
Further findings
Prognostic factors, non-survivors vs survivors
- Pediatric Index of Mortality (PIM2) higher among non-survivors
- Splenomegaly and leukopenia at PICU admission more common in non-survivors
- Multiple organ failure and acute renal failure during PICU stay more common in non-survivors
- Platelet counts significantly lower in patients who died, only at T2 (highest ferritin)
- Ferritin consistently elevated in non-survivors throughout PICU stay
Original publication
Critical care outcomes of hemophagocytic lymphohistiocytosis in children: a single centre retrospective observational study. Pediatr Rheumatol Online J. Published 6 July 2026. doi:10.
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