ArticleFrontiers in immunology2025
Macrophage activation syndrome successfully treated with eculizumab and emapalumab: a case report.
Article in Frontiers in immunology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.
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Who cites it
2 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Real-world use of emapalumab in hemophagocytic lymphohistiocytosis: a scoping review of published evidence.Frontiers in pharmacology · 2026Pooled it
- Low-dose emapalumab treatment in refractory macrophage activation syndrome secondary to adult onset still's disease/systemic lupus erythematosus: insights from nine cases.Frontiers in immunology · 2025Article
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Authors and funding
11 authors.
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Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, often referred to as macrophage activation syndrome (MAS) in the context of autoimmune disease-induced forms. We report the case of a 41-year-old woman with a previous diagnosis of Crohn's disease complicated by dermatomyositis, who was admitted in our hospital for the acute onset of fever, pancytopenia, and disseminated intravascular coagulation (DIC). The laboratory findings documented hyperferritinemia, hypertransaminasemia, increased lactate-dehydrogenase (LDH), hypertriglyceridemia, and elevation of inflammatory indices, along with complement consumption. MAS was confirmed by examination of the bone marrow. Consequently, the patient was treated with high doses of glucocorticoids, subcutaneous anakinra, and intravenous immunoglobulin (IVIg). Due to the persistence of signs of thrombotic microangiopathy, we started therapy with eculizumab which stabilized the patient without improvement, so we added emapalumab, resulting in clinical improvement and normalization of blood tests.
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