Mast Cell Activation Syndromes: Collegium Internationale Allergologicum Update 2022

Peter Valent, Karin Hartmann, Patrizia Bonadonna, Marek Niedoszytko, Massimo Triggiani, Michel Arock, Knut Brockow

Research output: Contribution to journalReview articlepeer-review

29 Scopus citations

Abstract

Mast cell activation syndromes (MCASs) are defined by systemic severe and recurrent mast cell activation, usually in form of anaphylaxis, a substantial, event-related increase of the serum tryptase level beyond the individual's baseline and a response of the symptomatology to drugs directed against mast cells, mast cell-derived mediators, or mediator effects. A number of predisposing genetic conditions, underlying allergic and other hypersensitivity states, and related comorbidities can contribute to the clinical manifestation of MCASs. These conditions include hereditary alpha tryptasemia, mastocytosis with an expansion of clonal KIT-mutated mast cells, atopic diathesis, and overt IgE-dependent and IgE-independent allergies. Several of these conditions have overlapping definitions and diagnostic criteria and may also develop concomitantly in the same patient. However, although criteria and clinical features overlap, each of these conditions is characterized by a unique constellation of variables and diagnostic criteria. Since two, three, or more conditions can coexist in the same patient, with obvious clinical implications, it is of crucial importance to diagnose the variant of MCAS precisely and to take all accompanying, underlying and potentially complicating conditions, and comorbidities into account when establishing the management plan. Indeed, most of these patients require multidisciplinary investigations and only a personalized treatment approach can lead to an optimal management plan providing an optimal quality of life and low risk of anaphylaxis.

Original languageEnglish
Pages (from-to)693-705
Number of pages13
JournalInternational Archives of Allergy and Immunology
Volume183
Issue number7
DOIs
StatePublished - 1 Jul 2022

Keywords

  • Classification
  • Diagnostic MCAS criteria
  • IgE
  • Mast cell activation syndrome (MCAS)
  • Mast cells
  • Tryptase

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