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Clinical Approach to Mast Cell Activation Syndromes: A Practical Overview

Matito A1, Escribese MM2, Longo N3, Mayorga C4, Luengo-Sánchez O5, Pérez-Gordo M6, Matheu V7, Labrador-Horrillo M5, Pascal M8, Seoane-Reula ME9, on behalf of the Comité de Inmunología de la Sociedad Española de Alergología e Inmunología Clínica (SEAIC).

1Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Toledo
2Department of Basic Medical Sciences, Faculty of Medicine, San Pablo CEU University, Madrid
3Allergy Department, Hospital Universitario de Araba, Osakidetza, Vitoria
4Allergy Research Group, Hospital Regional Universitario de Málaga, Instituto de Investigación Biomédica de Málaga-IBIMA, ARADyAL, Málaga
5Allergy Department, Vall d’Hebron Hospital Universitari, Vall d’Hebron Institut de Recerca (VHIR), Universitat Autònoma de Barcelona (UAB), ARADyAL research network, Instituto de Salud Carlos III (ISCIII), Barcelona
6Basic Medical Science Department, Faculty of Medicine, CEU San Pablo University, ARADyAL, Madrid
7IDP and Allergy Department, Hospital Universitario de Canarias, Tenerife
8Immunology Department, CDB, Hospital Clínic de Barcelona; IDIBAPS, Universitat de Barcelona; ARADyAL research network, Instituto de Salud Carlos III (ISCIII), Barcelona
9Pediatric Allergy and Immunology Unit, Hospital General Universitario Gregorio Marañón, Madrid

J Investig Allergol Clin Immunol 2021; Vol. 31(6)
doi: 10.18176/jiaci.0675

The diagnosis of mast cell activation syndromes (MCAS) is defined by 3 criteria: 1) typical clinical signs and symptoms of acute, recurrent (episodic) and systemic mast cell activation (MCA), 2) increase in tryptase level to plus 20 % + 2 ng/ml within a 1–4 hours after onset of the acute crisis, 3) response of MCA symptoms to antimediator therapy. Classification of MCAS requires applying highly sensitive and specific methodological approaches for assessing clonal bone marrow mast cells (BM MCs) at low frequencies. The Spanish Network on Mastocytosis (REMA) score is successfully used as predictive model for selecting MCAS candidates for BM studies based on a high probability of having an underlying clonal mast cell disorder (c-MCD). In this article, we propose a diagnostic algorithm and focus in the practical evaluation and management of patients with suspected MCAS.

Key words: Anaphylaxis, Antimediator therapy, Mast cell activation syndrome, Mast cell mediators release related symptoms, Tryptase