Recognizing and Managing Hemophagocytic Lymphohistiocytosis in Adults in the Intensive Care Unit - 22/05/26

Doi : 10.1016/j.ccc.2026.01.001

Bita Shakoory, MD ^a, Irini Sereti, MD, PhD ^b, Joseph M. Rocco, MD ^b,^⁎

^a Translational Autoinflammatory Diseases Section, National Institute of Allergy and Infectious Diseases, Bethesda, USA

^b Laboratory of Immunoregulation and Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, USA

^∗ Corresponding author. National Institute of Allergy and Infectious Disease, National Institutes of Health, NIH Building 10, Room 11B-15, 10 Center Drive, Bethesda, MD 20892. National Institute of Allergy and Infectious Disease National Institutes of Health NIH Building 10 Room 11B-15 10 Center Drive Bethesda MD 20892

Résumé

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.

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Keywords : Hemophagocytic lymphohistiocytosis, Hyperinflammatory syndromes, Intensive care, Sepsis, Multi-organ dysfunction