Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome.
Diagnostic algorithm
KIT D816V
MCAS
Mast cells
Tryptase
Journal
The journal of allergy and clinical immunology. In practice
ISSN: 2213-2201
Titre abrégé: J Allergy Clin Immunol Pract
Pays: United States
ID NLM: 101597220
Informations de publication
Date de publication:
04 2019
04 2019
Historique:
received:
04
12
2018
revised:
31
12
2018
accepted:
05
01
2019
pubmed:
10
2
2019
medline:
25
7
2020
entrez:
10
2
2019
Statut:
ppublish
Résumé
Mast cell activation (MCA) accompanies diverse physiologic and pathologic processes and is one of the more frequently encountered conditions in medicine. MCA-related symptoms are usually mild and often transient. In such cases, histamine receptor blockers and other mediator-targeting drugs can usually control MCA. In severe cases, an MCA syndrome (MCAS) may be diagnosed. However, overt MCAS is an unusual condition, and many patients referred because of suspected MCAS are diagnosed with other diseases (autoimmune, neoplastic, or infectious) unrelated to MCA or suffer from MCA-related (eg, allergic) disorders and/or comorbidities without fulfilling criteria of an overt MCAS. These considerations are important as more and more patients are informed that they may have MCA or even MCAS without completing a thorough medical evaluation. In fact, in several instances, symptoms are misinterpreted as MCA/MCAS, and other clinically relevant conditions are not thoroughly pursued. The number of such referrals is increasing. To avoid such unnecessary referrals and to prevent misdiagnoses, we here propose a diagnostic algorithm through which a clinically relevant (systemic) MCA can be suspected and MCAS can subsequently be documented or excluded. In addition, the algorithm proposed should help guide the investigating care providers to consider the 2 principal diagnoses that may underlie MCAS, namely, severe allergy and systemic mastocytosis accompanied by severe MCA. Although validation is required, we anticipate that this algorithm will facilitate the management of patients with suspected MCAS.
Identifiants
pubmed: 30737190
pii: S2213-2198(19)30056-X
doi: 10.1016/j.jaip.2019.01.006
pmc: PMC6643056
mid: NIHMS1518625
pii:
doi:
Substances chimiques
KIT protein, human
EC 2.7.10.1
Proto-Oncogene Proteins c-kit
EC 2.7.10.1
Tryptases
EC 3.4.21.59
Types de publication
Journal Article
Research Support, N.I.H., Intramural
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
1125-1133.e1Subventions
Organisme : Intramural NIH HHS
ID : ZIA AI001091-08
Pays : United States
Informations de copyright
Copyright © 2019 American Academy of Allergy, Asthma & Immunology. All rights reserved.
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