94% of researchers rate our articles as excellent or good
Learn more about the work of our research integrity team to safeguard the quality of each article we publish.
Find out more
CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1555415
Macrophage activation syndrome successfully treated with eculizumab and emapalumab: a case report
Provisionally accepted
Paola Faggioli 
Marianna Galeazzi * Carlotta Ferrari Francesca Capelli Chiara Marchesi Lucia Marchionni 
Laura Castelnovo Antonio Tamburello Cristina Campidelli Antonino Mazzone *- ASST Ovest Milanese, Legnano, Italy
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-years-old women with a previous diagnosis of Crohn's disease complicated by dermatomyositis, who was admitted to our Hospital for the acute onset of fever, pancytopenia and disseminated intravascular coagulation (DIC). Laboratory findings documented hyperferritinemia, hypertransaminasemia, increased lactate-dehydrogenase (LDH), hypertriglyceridemia, 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 wich stabilized the patient without improvement, so we added emapalumab resulting in clinical improvement and normalization of blood tests.
Keywords: Hemophagocytic lymphohistiocytosis (HLH), Macrophage Activation Syndrome (MAS), Eculizumab, Emapalumab, Thrombotic microangiopathy (TMA)
Received: 04 Jan 2025; Accepted: 20 Feb 2025.
Copyright: © 2025 Faggioli, Galeazzi, Ferrari, Capelli, Marchesi, Marchionni, Castelnovo, Tamburello, Campidelli and Mazzone. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Marianna Galeazzi, ASST Ovest Milanese, Legnano, Italy
Antonino Mazzone, ASST Ovest Milanese, Legnano, Italy
Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.