Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review

Theo Gülen, Cem Akin, Patrizia Bonadonna, Frank Siebenhaar, Sigurd Broesby-Olsen, Knut Brockow, Marek Niedoszytko, Boguslaw Nedoszytko, Hanneke N.G. Oude Elberink, Joseph H. Butterfield, Wolfgang R. Sperr, Ivan Alvarez-Twose, Hans Peter Horny, Karl Sotlar, Juliana Schwaab, Mohamad Jawhar, Roberta Zanotti, Gunnar Nilsson, Jonathan J. Lyons, Melody C. CarterTracy I. George, Olivier Hermine, Jason Gotlib, Alberto Orfao, Massimo Triggiani, Andreas Reiter, Karin Hartmann, Mariana Castells, Michel Arock, Lawrence B. Schwartz, Dean D. Metcalfe, Peter Valent

Research output: Contribution to journalArticlepeer-review

33 Scopus citations

Abstract

In recent years, knowledge about mechanisms underlying mast cell activation (MCA) and accumulation in various pathologic conditions increased substantially. In addition, criteria and a classification of MCA syndromes (MCASs) have been set forth. MCAS is defined by typical clinical symptoms, a substantial increase in serum tryptase level during an attack over the patient's baseline tryptase, and a response of the symptoms to drugs targeting mast cells, mediator production, and/or mediator effects. Alternative diagnostic criteria of MCAS have also been suggested, but these alternative criteria often lack specificity and validation. In this report, we critically review the contemporary literature relating to MCAS and compare the specificity, sensitivity, and strength of MCAS-related parameters within proposals to diagnose and classify MCAS and its variants. Furthermore, we highlight the need to apply specific consensus criteria in the evaluation and classification of MCAS in individual patients. This is an urgent and important medical necessity because as an increasing number of patients are being given a misdiagnosis of MCAS based on nonspecific criteria, which contributes to confusion and frustration by patients and caregivers and sometimes may delay recognition and treatment of correct medical conditions that often turn out to be unrelated to MCA.

Original languageEnglish
Pages (from-to)3918-3928
Number of pages11
JournalJournal of Allergy and Clinical Immunology: In Practice
Volume9
Issue number11
DOIs
StatePublished - Nov 2021
Externally publishedYes

Keywords

  • Anaphylaxis
  • Hereditary alpha Tryptasemia
  • MCAS
  • Mast cells
  • Mastocytosis
  • Tryptase
  • Vienna consensus criteria

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