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Gabr JB, Liu E, Mian S, et al. Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still's disease: case report and review of the literature. Ann Transl Med. 2020;8(14):887doi: 10.21037/atm-20-3127.
Gabr, J. B., Liu, E., Mian, S., Pillittere, J., Bonilla, E., Banki, K., & Perl, A. (2020). Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still's disease: case report and review of the literature. Annals of translational medicine, 8(14), 887. https://doi.org/10.21037/atm-20-3127
Gabr, Jihad Ben, et al. "Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still's disease: case report and review of the literature." Annals of translational medicine vol. 8,14 (2020): 887. doi: https://doi.org/10.21037/atm-20-3127
Gabr JB, Liu E, Mian S, Pillittere J, Bonilla E, Banki K, Perl A. Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still's disease: case report and review of the literature. Ann Transl Med. 2020 Jul;8(14):887. doi: 10.21037/atm-20-3127. PMID: 32793731; PMCID: PMC7396773.
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Ann Transl Med. 2020 Jul;8(14):887. doi: 10.21037/atm-20-3127.

Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still's disease: case report and review of the literature.

Annals of translational medicine

Jihad Ben Gabr, Eric Liu, Sundus Mian, Julie Pillittere, Eduardo Bonilla, Katalin Banki, Andras Perl

Affiliations

  1. Department of Medicine, Upstate Medical University, College of Medicine, Syracuse, NY, USA.
  2. Department of Pathology, State University of New York, College of Medicine, Syracuse, NY, USA.
  3. Department of Microbiology and Immunology, Upstate Medical University, College of Medicine, Syracuse, NY, USA.
  4. Department of Biochemistry and Molecular Biology, Upstate Medical University, College of Medicine, Syracuse, NY, USA.

PMID: 32793731 PMCID: PMC7396773 DOI: 10.21037/atm-20-3127

Abstract

Here, we present a 22-year-old female patient with adult-onset Still's disease (AOSD) who was newly diagnosed in the setting of secondary macrophage activation syndrome (MAS), a rare, life-threatening inflammatory disease with 50% mortality due to multi-organ failure. She met the diagnostic criteria of AOSD and MAS, while genetic testing excluded primary causes of MAS. She had high fevers, anemia, thrombocytopenia, splenomegaly, hematophagocytosis, and elevated serum ferritin (37,950 ng/mL) and CD25 levels (11,870 pg/mL), which remained unresponsive to corticosteroids and anakinra. Her serum interferon gamma (IFN-γ) levels were elevated (7 pg/mL). She was markedly responsive to IFN-γ blockade with emapalumab that eliminated her fevers and all MAS-associated laboratory abnormalities. This report provides initial evidence for therapeutic efficacy for IFN-γ blockade in AOSD and secondary MAS.

2020 Annals of Translational Medicine. All rights reserved.

Keywords: Macrophage activation syndrome (MAS); adult onset Still’s disease (AOSD); emapalumab; hemophagocytic lymphohistiocytosis (HLH); interferon gamma

Conflict of interest statement

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/atm-20-3127). The authors have no conflicts of interest to declare.

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