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

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 substa...

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Detalles Bibliográficos
Autores: Gülen, Theo, Akin, Cem, Bonadonna, Patrizia, Siebenhaar, Frank, Broesby-Olsen, Sigurd, Brockow, Knut, Niedoszytko, Marek, Nedoszytko, Boguslaw, Oude Elberink, Hanneke, Butterfield, Joseph H., Sperr, Wolfgang R., Álvarez-Twose, Iván, Horny, Hans-Peter, Sotlar, Karl, Schwaab, Juliana, Jawhar, Mohamad, Zanotti, Roberta, Nilsson, Gunnar, Lyons, Jonathan J., Carter, Melody C., George, Tracy I., Hermine, Olivier, Gotlib, Jason, Orfao, Alberto, Triggiani, Massimo, Reiter, Andreas, Hartmann, Karin, Castells, Mariana, Arock, Michel, Schwartz, Lawrence B., Metcalfe, Dean D., Valent, Peter
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2021
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/268013
Acceso en línea:http://hdl.handle.net/10261/268013
Access Level:acceso abierto
Palabra clave:Mast cells
MCAS
Tryptase
Hereditary alpha Tryptasemia
Mastocytosis
Anaphylaxis
Vienna consensus criteria
Descripción
Sumario: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.

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