Case Reports Journal Article Epublish 2 citations
PubMed: 40196116 DOI: 10.3389/fimmu.2025.1555415
Macrophage activation syndrome successfully treated with eculizumab and emapalumab: a case report.
Front Immunol 16:1555415
Authors: Paola Faggioli, Marianna Galeazzi, Carlotta Ferrari, Francesca Capelli, Chiara Marchesi, Lucia Marchionni, Laura Castelnovo, Antonio Tamburello, Eugenio Capparelli, Cristina Campidelli, Antonino Mazzone
Affiliations
- Department of Internal Medicine, Azienda Socio Sanitaria Territoriale (ASST) Ovest Milanese, Legnano, MI, Italy.
- Department of Pathological Anatomy, Azienda Socio Sanitaria Territoriale (ASST) Ovest Milanese, Legnano, MI, Italy.
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.
MeSH
Macrophage Activation, Macrophages, Immune System Diseases, Immunoglobulins, Intravenous, Macrophage Activation Syndrome