Plain language summary: emapalumab therapy for patients with rheumatologic conditions and hemophagocytic lymphohistiocytosis

This Plain Language Summary of Publication article (PLSP) from Immunotherapy explains hemophagocytic lymphohistiocytosis (HLH), a rare condition characterized by immune system overactivation leading to dangerous inflammation that can damage organs and be fatal if untreated. It describes how interferon-gamma (IFNγ) protein overproduction drives HLH’s hyperinflammation and introduces emapalumab, a medication that blocks IFNγ activity. It references the REAL-HLH study, which examined emapalumab’s effectiveness in clinical practice for U.S. patients, focusing specifically on results from patients with rheumatologic disease-associated HLH.

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This PLSP is based on an article called ‘Emapalumab Treatment in Patients With Rheumatologic Disease-Associated Hemophagocytic Lymphohistiocytosis in the United States: A Retrospective Medical Chart Review Study’ and was published in Arthritis & Rheumatology.

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• emapalumab
• hemophagocytic lymphohistiocytosis
• plain language summary
• REAL-HLH
• rheumatologic disease–associated hemophagocytic lymphohistiocytosis