Macrophage activation syndrome: A diagnostic challenge (Review).
diagnosis score
hemophagocytosis
inflammatory syndrome
macrophage activation syndrome
pancytopenia
Journal
Experimental and therapeutic medicine
ISSN: 1792-1015
Titre abrégé: Exp Ther Med
Pays: Greece
ID NLM: 101531947
Informations de publication
Date de publication:
Aug 2021
Aug 2021
Historique:
received:
26
03
2021
accepted:
26
04
2021
entrez:
14
7
2021
pubmed:
15
7
2021
medline:
15
7
2021
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 34257717
doi: 10.3892/etm.2021.10336
pii: ETM-0-0-10336
pmc: PMC8243343
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
904Informations de copyright
Copyright: © Bojan et al.
Déclaration de conflit d'intérêts
The authors declare that they have no competing interests.
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