Clinical Approach to Mast Cell Activation Syndrome: A Practical Overview

Autor: Cristobalina Mayorga, María M. Escribese, Victor Matheu, Marina Perez-Gordo, A Matito, M E Seoane-Reula, Mariona Pascal, O Luengo-Sánchez, Moises Labrador-Horrillo, N Longo
Rok vydání: 2021
Předmět:
Zdroj: Journal of Investigational Allergy and Clinical Immunology. 31:461-470
ISSN: 1018-9068
DOI: 10.18176/jiaci.0675
Popis: The diagnosis of mast cell activation syndrome (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 >20% + 2 ng/mL within 1-4 hours after onset of the acute crisis; and (3) response of MCA symptoms to antimediator therapy. Classification of MCAS requires highly sensitive and specific methodological approaches for the assessment of clonal bone marrow mast cells at low frequencies. The Spanish Network on Mastocytosis score has been used successfully as a predictive model for selecting MCAS candidates for bone marrow studies based on a high probability of an underlying clonal mast cell disorder. In this article, we propose a diagnostic algorithm and focus on the practical evaluation and management of patients with suspected MCAS. Key words: Anaphylaxis. Antimediator therapy. Mast cell activation syndrome. Mast cell mediator release–related symptoms. Tryptase.
Databáze: OpenAIRE