Anaphylaxis and Mast Cell Disorders - PubMed (original) (raw)
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Anaphylaxis and Mast Cell Disorders
Theo Gülen et al. Immunol Allergy Clin North Am. 2022 Feb.
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Abstract
There is strong evidence of an association between severe anaphylaxis, especially hymenoptera venom induced, and mast cell (MC) disorders. It has been thought that intrinsic abnormalities in MCs, including the presence of the activating KIT D816V mutation in mastocytosis or of genetic trait, hereditary alpha-tryptasemia, may influence susceptibility to severe anaphylaxis. This article evaluates the potential mechanisms leading to severe MC activation, as well as the differential diagnosis of and range of symptoms attributable to MC mediator release. Also, we offer a global classification for disorders related to MC activation.
Keywords: Anaphylaxis; D816 V mutation; Hereditary alpha-tryptasemia; Mast cell activation syndromes; Mast cell hyperreactivity; Mastocytosis; Tryptase.
Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.
Conflict of interest statement
Disclosure T. Gülen has received lecture fees from Thermo Fisher. C. Akin has received consultancy fees from Blueprint Medicines and Novartis and has a patent for LAD2 cells.
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