Anaphylaxis and Mast Cell Disorders - 23/11/21

Doi : 10.1016/j.iac.2021.09.007

Theo Gülen, MD, PhD ^a,^b,^c,^⁎ , Cem Akin, MD, PhD ^d
^a Department of Respiratory Medicine and Allergy, Karolinska University Hospital, Huddinge
^b Department of Medicine Solna, Immunology and Allergy Unit, Karolinska Institutet and Karolinska University Hospital
^c Mastocytosis Centre Karolinska, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden
^d Department of Internal Medicine, Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, MI, USA

^∗Correspondence author. Department of Respiratory Medicine and Allergy, K85, Karolinska University Hospital Huddinge, Stockholm SE-141 86, Sweden.Department of Respiratory Medicine and AllergyK85, Karolinska University Hospital HuddingeStockholmSE-141 86Sweden

Résumé

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.

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Keywords : Anaphylaxis, Mastocytosis, Mast cell hyperreactivity, D816 V mutation, Tryptase, Mast cell activation syndromes, Hereditary alpha-tryptasemia