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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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
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spelling Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical ReviewGülen, TheoAkin, CemBonadonna, PatriziaSiebenhaar, FrankBroesby-Olsen, SigurdBrockow, KnutNiedoszytko, MarekNedoszytko, BoguslawOude Elberink, HannekeButterfield, Joseph H.Sperr, Wolfgang R.Álvarez-Twose, IvánHorny, Hans-PeterSotlar, KarlSchwaab, JulianaJawhar, MohamadZanotti, RobertaNilsson, GunnarLyons, Jonathan J.Carter, Melody C.George, Tracy I.Hermine, OlivierGotlib, JasonOrfao, AlbertoTriggiani, MassimoReiter, AndreasHartmann, KarinCastells, MarianaArock, MichelSchwartz, Lawrence B.Metcalfe, Dean D.Valent, PeterMast cellsMCASTryptaseHereditary alpha TryptasemiaMastocytosisAnaphylaxisVienna consensus criteriaIn 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.ElsevierConsejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]2022202220212022info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Publisher's versioninfo:eu-repo/semantics/publishedVersionhttp://hdl.handle.net/10261/268013reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Ingléshttp://dx.doi.org/10.1016/j.jaip.2021.06.011Síinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/2680132026-05-22T06:33:51Z
dc.title.none.fl_str_mv Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
title Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
spellingShingle Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
Gülen, Theo
Mast cells
MCAS
Tryptase
Hereditary alpha Tryptasemia
Mastocytosis
Anaphylaxis
Vienna consensus criteria
title_short Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
title_full Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
title_fullStr Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
title_full_unstemmed Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
title_sort Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review
dc.creator.none.fl_str_mv 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
author Gülen, Theo
author_facet 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
author_role author
author2 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
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]
dc.subject.none.fl_str_mv Mast cells
MCAS
Tryptase
Hereditary alpha Tryptasemia
Mastocytosis
Anaphylaxis
Vienna consensus criteria
topic Mast cells
MCAS
Tryptase
Hereditary alpha Tryptasemia
Mastocytosis
Anaphylaxis
Vienna consensus criteria
description 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.
publishDate 2021
dc.date.none.fl_str_mv 2021
2022
2022
2022
dc.type.none.fl_str_mv info:eu-repo/semantics/article
http://purl.org/coar/resource_type/c_6501
Publisher's version
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10261/268013
url http://hdl.handle.net/10261/268013
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv http://dx.doi.org/10.1016/j.jaip.2021.06.011
Sí
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv Elsevier
publisher.none.fl_str_mv Elsevier
dc.source.none.fl_str_mv reponame:DIGITAL.CSIC. Repositorio Institucional del CSIC
instname:Consejo Superior de Investigaciones Científicas (CSIC)
instname_str Consejo Superior de Investigaciones Científicas (CSIC)
reponame_str DIGITAL.CSIC. Repositorio Institucional del CSIC
collection DIGITAL.CSIC. Repositorio Institucional del CSIC
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,812429

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