Open Access

Macrophage activation syndrome: A diagnostic challenge (Review)

  • Authors:
    • Anca Bojan
    • Andrada Parvu
    • Iulia-Andrea Zsoldos
    • Tunde Torok
    • Anca Daniela Farcas
  • View Affiliations

  • Published online on: June 24, 2021     https://doi.org/10.3892/etm.2021.10336
  • Article Number: 904
  • Copyright: © Bojan et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Macrophage activation syndrome (MAS) represents an acute and severe inflammatory syndrome, idiopathic (primary) or secondary to infections, rheumatic diseases, malignancies, or drugs. MAS is underdiagnosed, being confused with sepsis, adverse effects of anti‑arthritic drugs or exacerbated symptoms of evolving rheumatologic or infectious diseases. Because of the late diagnosis, most patients do not benefit from effective therapy, leading to death. Elucidation of valid early diagnostic criteria of MAS would be a particularly important step in reducing the mortality due to this pathology. Thus, the purpose of this review based on 40 studies centered on the diagnostic criteria of MAS. We detailed the main diagnostic criteria and the few diagnostic scores or sets of criteria that have been recently published. The criteria most frequently encountered in the literature include: Fever, hepatosplenomegaly, hyperferritinemia, hepatopathy, coagulopathy, thrombocytopenia, hypertriglyceridemia, decrease in erythrocyte sedimentation rate and bone marrow hemophagocytosis. The most elaborate diagnostic score will result following an ongoing international project and consensus, the Delphi International Survey.
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August-2021
Volume 22 Issue 2

Print ISSN: 1792-0981
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Spandidos Publications style
Bojan A, Parvu A, Zsoldos I, Torok T and Farcas AD: Macrophage activation syndrome: A diagnostic challenge (Review). Exp Ther Med 22: 904, 2021
APA
Bojan, A., Parvu, A., Zsoldos, I., Torok, T., & Farcas, A.D. (2021). Macrophage activation syndrome: A diagnostic challenge (Review). Experimental and Therapeutic Medicine, 22, 904. https://doi.org/10.3892/etm.2021.10336
MLA
Bojan, A., Parvu, A., Zsoldos, I., Torok, T., Farcas, A. D."Macrophage activation syndrome: A diagnostic challenge (Review)". Experimental and Therapeutic Medicine 22.2 (2021): 904.
Chicago
Bojan, A., Parvu, A., Zsoldos, I., Torok, T., Farcas, A. D."Macrophage activation syndrome: A diagnostic challenge (Review)". Experimental and Therapeutic Medicine 22, no. 2 (2021): 904. https://doi.org/10.3892/etm.2021.10336