Recognizing and Managing Hemophagocytic Lymphohistiocytosis in Adults in the Intensive Care Unit

Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammatory syndrome increasingly recognized across age groups. This syndrome is driven by pathologic interferon-γ production, which leads to a self-sustaining positive feedback loop resulting in multi-organ dysfunction with a high mortality. Early recognition is essential, and clinical evaluation should prioritize identifying any predisposing diseases and acute triggers. Treatment requires a multi-faceted approach, including dampening the hyperinflammation, eliminating acute triggers and infectious complications, and optimizing management of all underlying predispositions. Novel prognostic markers (C-X-C motif ligand-9), and cytokine-directed therapies (ruxolitinib, emapalumab) show promise to help improve outcomes of patients with these complex hyperinflammatory syndromes in the future.