CO13 | Macrophage activation syndrome successfully treated with eculizumab and emapalumab: a case report
Year: 2025
Type: article
Study design: Case Report
Abstract: Premises: Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome. If it occurs in autoimmune diseases the term macrophage activation syndrome is used. Infections represent a trigger of acute episode. Description of the Case report: A 45-year-old women suffering from Crohn’s disease complicated by polimyositis, treated with DMARDs and steroids and, since Sept. 2023 with infliximab, in Dec.2023 was recovered for fever. Blood tests showed: pancytopenia, hyperferritinemia, hypertransaminasemia, increase in LDH, triglycerides, CPK and inflammation indices; complement consumption. MAS was confirmed by examination of the bone marrow. We documented possible recent EBV and M. pneumoniae infections. Therapy with high dose streoids, anakinra and IVIg was established; she was treated with antibiotics, antivirals and antifungals. Due to the persistence of signs of thrombotic microangiopathy we decided to start association therapy with eculizumab, a mAb designed to attach to the C5 complement protein, with stabilization but missed improvement, so the patient was treated with emapalumab, with clinical improvement and normalization of LDH, complement and fibrinogen. Conclusions: The treatment of HLH consists of glucocorticoids, anakinra and IVIg. Treatment of the underlying disease should be carried out in parallel. Emapalumab is the first targeted therapy approved by the US FDA for primary pediatric HLH. Ours is one of the first reports of the use of Emapalumab in adult patients: this drug has proven to be effective for a pathology burdened by a very high mortality rate.
Source: Italian Journal of Medicine
Authors: Vo Van Kha, Tran Thi Huong Ly, Pham Thi Thanh Hoa, Vo Huynh Nhu, Damas Hendriansyah, Nusratuddin Abdullah, Rudy B. Leonardy, Mardiah Tahir, Samrichard Rambulangi, Firdaus Hamid, Filomena Pietrantonio, Vera Kopsaj, Francesco Rosiello, Aurora Tomassoni, Luca Moriconi, Donatella Padua, Kiki Rizki Amelia, Efendi Lukas, Nurbani Bangsawan, Isharyah Sunarno, Retno Budiarti Farid, Sriwijaya Qadar, Rabab H. Elshaikh, Ashok Kumar Sah, Abdelrahman Mohamed Abukanna, Abdulrahman Algarni, Ammar Abdelmola, Hussam Ali Osman, Tagwa Yousif Elsayed Yousif, Elryah I. Ali, Hiba Ali, Ayman Hussein Alfeel, Thanh Long Nguyen, Victor Storm, Lam Toi Phung, Cong Thien Le, Thi Thu Ha Le, Thi Hoa Nguyen, Son Tung Vu, Thien Thang Tran, Minh Hanh Vu, Van Tuan Nguyen, Francesco Ventrella, Anna Belfiore, Antonio Greco, Franco Mastroianni, Salvatore Lenti, Domenico Ruggiero, Michele Cannone, Vincenzo Ostilio Palmieri, Massimo Errico, Giuseppe Mancia, Guido Grassi, Sverre E. Kjeldsen
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4413772326