Conclusions
Children with HLH who have a pSOFA score of ≥ 8, or are younger than 3
years, are at a higher risk of relapsed or refractory disease. These
findings suggest a new application for the pSOFA score, which was
recently proposed as a severity grading tool in consensus-based
guidelines from the Histiocyte Society. Further evaluation of management
strategies in this context is warranted.