A Puzzling Mast Cell Trilogy: Anaphylaxis, MCAS, and Mastocytosis.
KIT D816V
MCAS
anaphylaxis
atopy
hereditary alpha-tryptasemia
mastocytosis
mediator-release symptoms
tryptase
Journal
Diagnostics (Basel, Switzerland)
ISSN: 2075-4418
Titre abrégé: Diagnostics (Basel)
Pays: Switzerland
ID NLM: 101658402
Informations de publication
Date de publication:
25 Oct 2023
25 Oct 2023
Historique:
received:
18
09
2023
revised:
10
10
2023
accepted:
12
10
2023
medline:
14
11
2023
pubmed:
14
11
2023
entrez:
14
11
2023
Statut:
epublish
Résumé
Our knowledge of biology and mast cell (MC) function, as well as disorders associated with the pathologic activation of MCs, has evolved over the last few decades. Anaphylaxis, mast cell activation syndrome (MCAS), and mastocytosis are interrelated yet distinct conditions within the spectrum of mast cell activation disorders. Nevertheless, all three conditions can co-exist in one and the same patient, as pathologic MC activation is the key finding in all three. When mediator release is excessive and involves multiple systems, anaphylaxis and MCAS may occur. Furthermore, mastocytosis is a clonal disorder of MCs and often presents with anaphylaxis and MCAS. Nevertheless, in some cases, even the proliferative and accumulative features of MCs in mastocytosis can account for symptoms and disease progression. In each case, diagnosis can be only made when the diagnostic consensus criteria are fulfilled. The current article aims to provide a concise clinical update and pinpoint the main difficulties in diagnosing these puzzling disorders of MCs in medical practice.
Identifiants
pubmed: 37958203
pii: diagnostics13213307
doi: 10.3390/diagnostics13213307
pmc: PMC10647312
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Subventions
Organisme : The Swedish Society of Medicine, Sweden
ID : n/a
Organisme : Konsul T.H.C. Bergh Foundation, Sweden
ID : n/a
Organisme : The regional agreement on medical training and clinical research (ALF) between Stockholm County Council and Karolinska Institutet, Stockholm, Sweden.
ID : n/a
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