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Significance Adult secondary hemophagocytic lymphohistiocytosis (sHLH) is a fulminant hyperinflammatory syndrome with mortality exceeding 80% in spite of state-of-the-art medical care. The pathogenesis of sHLH, unlike primary HLH, which is primarily genetic, is poorly understood. Here we describe a model of sHLH that recapitulates many aspects of human sHLH. We found that the hyperinflammation in this model is macrophage intrinsic and identified a unique transcriptional profile associated with sHLH macrophages that was also present in patients with sHLH. We also found that these hyperinflammatory macrophages were highly dependent on glycolytic metabolism. Consequently, we found that administration of the glycolysis inhibitor 2-deoxyglucose was sufficient to rescue animals with sHLH by significantly suppressing the inflammatory response.

Specific sequences of infectious challenge lead to secondary hemophagocytic lymphohistiocytosis-like disease in mice