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• W4281485518 abstract "Mast cell activation (MCA) is common and occurs in a number of pathologic conditions, including IgE-dependent and independent allergic reactions, atopic disorders, autoimmune processes, and mastocytosis. In a subset of patients, no underlying disease and no known trigger of MCA are found. When the symptoms are severe, systemic, and recurrent, and accompanied by a diagnostic increase in the serum tryptase level or other mast cell mediators, an MCA syndrome (MCAS) may be diagnosed. In these patients, the symptoms typically respond to drugs suppressing MCA, mediator production in mast cells, or mediator effects. In each case, diagnostic consensus criteria must be fulfilled to diagnose MCAS. In other patients, MCA may be local, less severe, or less acute, or may be suspected but not confirmed, so that the diagnostic criteria of MCAS are not fulfilled. In these patients, it may be difficult to prove MCA, for example, by measuring multiple mast cell mediators or basophil activation, the latter as a surrogate of IgE-dependent hypersensitivity. However, validated diagnostic criteria for implicating suspected MCA behind such conditions are lacking, even if some of these conditions have recently been assigned to an International Classification of Diseases-10-Clinical Modification code (ICD-10-CM). In this article, we discuss diagnostic features and criteria and propose a ICD-10-CM–adjusted classification for disorders associated with MCA, herein referred to as MCA disorders (MCADs), with special emphasis on the delineation between confirmed MCAS, MCAD not fulfilling MCAS criteria, and suspected MCAD that is not present. In addition, we discuss the discrimination between overt MCAD and predisposing conditions, such as atopic states, mastocytosis, and hereditary alpha tryptasemia. Mast cell activation (MCA) is common and occurs in a number of pathologic conditions, including IgE-dependent and independent allergic reactions, atopic disorders, autoimmune processes, and mastocytosis. In a subset of patients, no underlying disease and no known trigger of MCA are found. When the symptoms are severe, systemic, and recurrent, and accompanied by a diagnostic increase in the serum tryptase level or other mast cell mediators, an MCA syndrome (MCAS) may be diagnosed. In these patients, the symptoms typically respond to drugs suppressing MCA, mediator production in mast cells, or mediator effects. In each case, diagnostic consensus criteria must be fulfilled to diagnose MCAS. In other patients, MCA may be local, less severe, or less acute, or may be suspected but not confirmed, so that the diagnostic criteria of MCAS are not fulfilled. In these patients, it may be difficult to prove MCA, for example, by measuring multiple mast cell mediators or basophil activation, the latter as a surrogate of IgE-dependent hypersensitivity. However, validated diagnostic criteria for implicating suspected MCA behind such conditions are lacking, even if some of these conditions have recently been assigned to an International Classification of Diseases-10-Clinical Modification code (ICD-10-CM). In this article, we discuss diagnostic features and criteria and propose a ICD-10-CM–adjusted classification for disorders associated with MCA, herein referred to as MCA disorders (MCADs), with special emphasis on the delineation between confirmed MCAS, MCAD not fulfilling MCAS criteria, and suspected MCAD that is not present. In addition, we discuss the discrimination between overt MCAD and predisposing conditions, such as atopic states, mastocytosis, and hereditary alpha tryptasemia. INFORMATION FOR CATEGORY 1 CME CREDITCredit can now be obtained, free for a limited time, by reading the review articles in this issue. Please note the following instructions.Method of Physician Participation in Learning Process: The core material for these activities can be read in this issue of the Journal or online at the JACI: In Practice Web site: www.jaci-inpractice.org/. The accompanying tests may only be submitted online at www.jaci-inpractice.org/. Fax or other copies will not be accepted.Date of Original Release: August 1, 2022. Credit may be obtained for these courses until July 31, 2023.Copyright Statement: Copyright © 2022-2024. All rights reserved.Overall Purpose/Goal: To provide excellent reviews on key aspects of allergic disease to those who research, treat, or manage allergic disease.Target Audience: Physicians and researchers within the field of allergic disease.Accreditation/Provider Statements and Credit Designation: The American Academy of Allergy, Asthma & Immunology (AAAAI) is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. The AAAAI designates this journal-based CME activity for 1.00 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.List of Design Committee Members: Peter Valent, MD, Karin Hartmann, MD, Patrizia Bonadonna, MD, Theo Gülen, MD, Knut Brockow, MD, Ivan Alvarez-Twose, MD, PhD, Olivier Hermine, MD, PhD, Marek Niedoszytko, MD, PhD, Melody C. Carter, MD, Gregor Hoermann, MD, PhD, Joseph H. Butterfield, MD, Jonathan J. Lyons, MD, Wolfgang R. Sperr, MD, Georg Greiner, MD, PhD, Karl Sotlar, MD, Hanneke C. Kluin-Nelemans, MD, PhD, Juliana Schwaab, MD, Magdalena Lange, MD, PhD, Tracy I. George, MD, Frank Siebenhaar, MD, Sigurd Broesby-Olsen, MD, Mohamad Jawhar, MD, Boguslaw Nedoszytko, PhD, Mariana Castells, MD, PhD, Alberto Orfao, MD, PhD, Jason Gotlib, MD, MS, Andreas Reiter, MD, Hans-Peter Horny, MD, Massimo Triggiani, MD, PhD, Michel Arock, PharmD, PhD, Dean D. Metcalfe, MD, MS, and Cem Akin, MD, PhD (authors); Michael Schatz, MD, MS (editor)Learning objectives: 1.To develop the ability to diagnose mast cell activation disorders (MCAD), including mast cell activation syndromes (MCAS) using diagnostic criteria and to distinguish between true MCAD/MCAS and MCAD/MCAS-like conditions.2.To understand the mechanisms of mast cell activation and recognize the impact of underlying predisposing and triggering conditions and pathologies.3.To acquire the ability to classify MCAS into MCAS variants, and to distinguish between MCAS, other MCAD, and unspecified MCAD where mast cell involvement is not always confirmed.4.To develop management skills for patients with MCAD and MCAS, including prevention and prophylaxis, basic anti-mediator-type therapy, and special forms of intervention.5.To understand the importance of multiple (and sometimes co-existing) predisposing and cooperating mast cell activation-inducing conditions and pathologies, recognize combined forms of MCAS, and develop skills to manage these combined and thus more severe MCAS patients by applying personalized medicine approaches.Recognition of Commercial Support: This CME has not received external commercial support.Disclosure of Relevant Financial Relationships with Commercial Interests: All authors and reviewers reported no relevant financial relationships.IntroductionMast cells (MCs) are key effector cells in IgE-dependent allergic disorders and other inflammatory conditions.1Metcalfe D.D. Mast cells and mastocytosis.Blood. 2008; 112: 946-956Crossref PubMed Scopus (415) Google Scholar, 2Galli S.J. Tsai M. IgE and mast cells in allergic disease.Nat Med. 2012; 18: 693-704Crossref PubMed Scopus (1104) Google Scholar, 3Theoharides T.C. Valent P. Akin C. Mast cells, mastocytosis, and related disorders.N Engl J Med. 2015; 373: 163-172Crossref PubMed Scopus (285) Google Scholar, 4Mukai K. Tsai M. Saito H. Galli S.J. Mast cells as sources of cytokines, chemokines, and growth factors.Immunol Rev. 2018; 282: 121-150Crossref PubMed Scopus (337) Google Scholar, 5Valent P. Akin C. Hartmann K. Nilsson G. Reiter A. Hermine O. et al.Mast cells as a unique hematopoietic lineage and cell system: from Paul Ehrlich’s visions to precision medicine concepts.Theranostics. 2020; 10: 10743-10768Crossref PubMed Scopus (50) Google Scholar, 6Galli S.J. Gaudenzio N. Tsai M. Mast cells in inflammation and disease: recent progress and ongoing concerns.Annu Rev Immunol. 2020; 38: 49-77Crossref PubMed Scopus (89) Google Scholar MCs exhibit high-affinity receptors for IgE (IgER = FcεRI) and produce inflammatory and vasoactive mediators, including histamine, tryptases, other proteases, prostaglandin D2 (PGD2), cysteinyl leukotrienes, and diverse chemokines and cytokines.1Metcalfe D.D. Mast cells and mastocytosis.Blood. 2008; 112: 946-956Crossref PubMed Scopus (415) Google Scholar, 2Galli S.J. Tsai M. IgE and mast cells in allergic disease.Nat Med. 2012; 18: 693-704Crossref PubMed Scopus (1104) Google Scholar, 3Theoharides T.C. Valent P. Akin C. Mast cells, mastocytosis, and related disorders.N Engl J Med. 2015; 373: 163-172Crossref PubMed Scopus (285) Google Scholar, 4Mukai K. Tsai M. Saito H. Galli S.J. Mast cells as sources of cytokines, chemokines, and growth factors.Immunol Rev. 2018; 282: 121-150Crossref PubMed Scopus (337) Google Scholar, 5Valent P. Akin C. Hartmann K. Nilsson G. Reiter A. Hermine O. et al.Mast cells as a unique hematopoietic lineage and cell system: from Paul Ehrlich’s visions to precision medicine concepts.Theranostics. 2020; 10: 10743-10768Crossref PubMed Scopus (50) Google Scholar, 6Galli S.J. Gaudenzio N. Tsai M. Mast cells in inflammation and disease: recent progress and ongoing concerns.Annu Rev Immunol. 2020; 38: 49-77Crossref PubMed Scopus (89) Google Scholar In physiologic states, several of these molecules are stored in the secretory granules of MCs. Some of these mediators, such as alpha tryptase, are released from MCs at a constant low rate under physiologic conditions, resulting in a low baseline serum concentration.5Valent P. Akin C. Hartmann K. Nilsson G. Reiter A. Hermine O. et al.Mast cells as a unique hematopoietic lineage and cell system: from Paul Ehrlich’s visions to precision medicine concepts.Theranostics. 2020; 10: 10743-10768Crossref PubMed Scopus (50) Google Scholar,7Schwartz L.B. Metcalfe D.D. Miller J.S. Earl H. Sullivan T. Tryptase levels as an indicator of mast-cell activation in systemic anaphylaxis and mastocytosis.N Engl J Med. 1987; 316: 1622-1626Crossref PubMed Scopus (665) Google Scholar,8Schwartz L.B. Sakai K. Bradford T.R. Ren S. Zweiman B. Worobec A.S. et al.The alpha form of human tryptase is the predominant type present in blood at baseline in normal subjects and is elevated in those with systemic mastocytosis.J Clin Invest. 1995; 96: 2702-2710Crossref PubMed Scopus (330) Google Scholar During an anaphylactic episode, cross-linking of FcεRI is followed by a massive and rapid release of granular mediators and enzymes from MCs.2Galli S.J. Tsai M. IgE and mast cells in allergic disease.Nat Med. 2012; 18: 693-704Crossref PubMed Scopus (1104) Google Scholar, 3Theoharides T.C. Valent P. Akin C. Mast cells, mastocytosis, and related disorders.N Engl J Med. 2015; 373: 163-172Crossref PubMed Scopus (285) Google Scholar, 4Mukai K. Tsai M. Saito H. Galli S.J. Mast cells as sources of cytokines, chemokines, and growth factors.Immunol Rev. 2018; 282: 121-150Crossref PubMed Scopus (337) Google Scholar, 5Valent P. Akin C. Hartmann K. Nilsson G. Reiter A. Hermine O. et al.Mast cells as a unique hematopoietic lineage and cell system: from Paul Ehrlich’s visions to precision medicine concepts.Theranostics. 2020; 10: 10743-10768Crossref PubMed Scopus (50) Google Scholar, 6Galli S.J. Gaudenzio N. Tsai M. Mast cells in inflammation and disease: recent progress and ongoing concerns.Annu Rev Immunol. 2020; 38: 49-77Crossref PubMed Scopus (89) Google Scholar In addition, during degranulation, cell surface and cytoplasmic membranes of MCs are rearranged, and lipid membrane–derived mediators are produced and secreted.2Galli S.J. Tsai M. IgE and mast cells in allergic disease.Nat Med. 2012; 18: 693-704Crossref PubMed Scopus (1104) Google Scholar, 3Theoharides T.C. Valent P. Akin C. Mast cells, mastocytosis, and related disorders.N Engl J Med. 2015; 373: 163-172Crossref PubMed Scopus (285) Google Scholar, 4Mukai K. Tsai M. Saito H. Galli S.J. Mast cells as sources of cytokines, chemokines, and growth factors.Immunol Rev. 2018; 282: 121-150Crossref PubMed Scopus (337) Google Scholar, 5Valent P. Akin C. Hartmann K. Nilsson G. Reiter A. Hermine O. et al.Mast cells as a unique hematopoietic lineage and cell system: from Paul Ehrlich’s visions to precision medicine concepts.Theranostics. 2020; 10: 10743-10768Crossref PubMed Scopus (50) Google Scholar, 6Galli S.J. Gaudenzio N. Tsai M. Mast cells in inflammation and disease: recent progress and ongoing concerns.Annu Rev Immunol. 2020; 38: 49-77Crossref PubMed Scopus (89) Google Scholar Basophils behave in a similar manner and can thus also contribute to allergic and other inflammatory reactions.9Falcone F.H. Knol E.F. Gibbs B.F. The role of basophils in the pathogenesis of allergic disease.Clin Exp Allergy. 2011; 41: 939-947Crossref PubMed Scopus (45) Google Scholar,10Reber L.L. Marichal T. Mukai K. Kita Y. Tokuoka S.M. Roers A. et al.Selective ablation of mast cells or basophils reduces peanut-induced anaphylaxis in mice.J Allergy Clin Immunol. 2013; 132: 881-888.e1-11Abstract Full Text Full Text PDF PubMed Scopus (76) Google Scholar However, in severe anaphylaxis, MCs are generally considered to be the primary cell involved in the resulting pathology.The capacity of MCs to release mediators of anaphylaxis in response to a specific trigger (agonist) depends on various circumstances and factors, including the genetic background of the patient, underlying pathology (disease), and the numbers and types of receptors and signaling molecules involved.11Peavy R.D. Metcalfe D.D. Understanding the mechanisms of anaphylaxis.Curr Opin Allergy Clin Immunol. 2008; 8: 310-315Crossref PubMed Scopus (134) Google Scholar, 12Metcalfe D.D. Peavy R.D. Gilfillan A.M. Mechanisms of mast cell signaling in anaphylaxis.J Allergy Clin Immunol. 2009; 124: 639-646Abstract Full Text Full Text PDF PubMed Scopus (209) Google Scholar, 13Kalesnikoff J. Galli S.J. Anaphylaxis: mechanisms of mast cell activation.Chem Immunol Allergy. 2010; 95: 45-66Crossref PubMed Scopus (53) Google Scholar, 14Valent P. Risk factors and management of severe life-threatening anaphylaxis in patients with clonal mast cell disorders.Clin Exp Allergy. 2014; 44: 914-920Crossref PubMed Scopus (30) Google Scholar, 15Lyons J.J. Chovanec J. O’Connell M.P. Liu Y. Šelb J. Zanotti R. et al.Heritable risk for severe anaphylaxis associated with increased alpha-tryptase-encoding germline copy number at TPSAB1.J Allergy Clin Immunol. 2021; 147: 622-632Abstract Full Text Full Text PDF PubMed Scopus (77) Google Scholar The severity of an anaphylactic reaction is further determined by the numbers of MCs involved, cytokine exposure, the nature and number of triggers and cofactors (eg, IgE-reactive allergens, nonsteroidals, and toxins such as those within venoms), the type and amount of IgE, the presence of comorbidities (eg, allergy, infectious diseases, or mastocytosis), epigenetic factors, and other patient-related variables (cardiovascular status, physical exercise, nutrition, alcohol, drugs).3Theoharides T.C. Valent P. Akin C. Mast cells, mastocytosis, and related disorders.N Engl J Med. 2015; 373: 163-172Crossref PubMed Scopus (285) Google Scholar,11Peavy R.D. Metcalfe D.D. Understanding the mechanisms of anaphylaxis.Curr Opin Allergy Clin Immunol. 2008; 8: 310-315Crossref PubMed Scopus (134) Google Scholar, 12Metcalfe D.D. Peavy R.D. Gilfillan A.M. Mechanisms of mast cell signaling in anaphylaxis.J Allergy Clin Immunol. 2009; 124: 639-646Abstract Full Text Full Text PDF PubMed Scopus (209) Google Scholar, 13Kalesnikoff J. Galli S.J. Anaphylaxis: mechanisms of mast cell activation.Chem Immunol Allergy. 2010; 95: 45-66Crossref PubMed Scopus (53) Google Scholar, 14Valent P. Risk factors and management of severe life-threatening anaphylaxis in patients with clonal mast cell disorders.Clin Exp Allergy. 2014; 44: 914-920Crossref PubMed Scopus (30) Google Scholar, 15Lyons J.J. Chovanec J. O’Connell M.P. Liu Y. Šelb J. Zanotti R. et al.Heritable risk for severe anaphylaxis associated with increased alpha-tryptase-encoding germline copy number at TPSAB1.J Allergy Clin Immunol. 2021; 147: 622-632Abstract Full Text Full Text PDF PubMed Scopus (77) Google Scholar, 16Bischoff S.C. Dahinden C.A. c-kit ligand: a unique potentiator of mediator release by human lung mast cells.J Exp Med. 1992; 175: 237-244Crossref PubMed Scopus (241) Google Scholar, 17Sperr W.R. Czerwenka K. Mundigler G. Müller M.R. Semper H. Klappacher G. et al.Specific activation of human mast cells by the ligand for c-kit: comparison between lung, uterus and heart mast cells.Int Arch Allergy Immunol. 1993; 102: 170-175Crossref PubMed Scopus (65) Google Scholar, 18Reber L.L. Hernandez J.D. Galli S.J. The pathophysiology of anaphylaxis.J Allergy Clin Immunol. 2017; 140: 335-348Abstract Full Text Full Text PDF PubMed Scopus (200) Google Scholar, 19Valenta R. Karaulov A. Niederberger V. Gattinger P. van Hage M. Flicker S. et al.Molecular aspects of allergens and allergy.Adv Immunol. 2018; 138: 195-256Crossref PubMed Scopus (57) Google Scholar, 20Sprinzl B. Greiner G. Uyanik G. Arock M. Haferlach T. Sperr W.R. et al.Genetic regulation of tryptase production and clinical impact: hereditary alpha tryptasemia, mastocytosis and beyond.Int J Mol Sci. 2021; 22: 2458Crossref PubMed Scopus (12) Google ScholarMC activation (MCA) occurs in a number of physiologic and pathologic states. Acute MCA accompanies IgE-dependent allergic reactions and may result in the clinical picture of anaphylaxis.2Galli S.J. Tsai M. IgE and mast cells in allergic disease.Nat Med. 2012; 18: 693-704Crossref PubMed Scopus (1104) Google Scholar,3Theoharides T.C. Valent P. Akin C. Mast cells, mastocytosis, and related disorders.N Engl J Med. 2015; 373: 163-172Crossref PubMed Scopus (285) Google Scholar,11Peavy R.D. Metcalfe D.D. Understanding the mechanisms of anaphylaxis.Curr Opin Allergy Clin Immunol. 2008; 8: 310-315Crossref PubMed Scopus (134) Google Scholar, 12Metcalfe D.D. Peavy R.D. Gilfillan A.M. Mechanisms of mast cell signaling in anaphylaxis.J Allergy Clin Immunol. 2009; 124: 639-646Abstract Full Text Full Text PDF PubMed Scopus (209) Google Scholar, 13Kalesnikoff J. Galli S.J. Anaphylaxis: mechanisms of mast cell activation.Chem Immunol Allergy. 2010; 95: 45-66Crossref PubMed Scopus (53) Google Scholar, 14Valent P. Risk factors and management of severe life-threatening anaphylaxis in patients with clonal mast cell disorders.Clin Exp Allergy. 2014; 44: 914-920Crossref PubMed Scopus (30) Google Scholar,19Valenta R. Karaulov A. Niederberger V. Gattinger P. van Hage M. Flicker S. et al.Molecular aspects of allergens and allergy.Adv Immunol. 2018; 138: 195-256Crossref PubMed Scopus (57) Google Scholar Severe or even life-threatening MCA may develop when (1) the burden of MCs is high, and/or (2) when most or all MCs are in a “hyperactivated” state and release all their mediators massively and instantly, and/or (3) when comorbidities such as cardiovascular or pulmonary diseases contribute substantially to an MCA event. When hypersensitivity reactions are severe, systemic, and recurrent, an MCA syndrome (MCAS) may be diagnosed.21Akin C. Valent P. Metcalfe D.D. Mast cell activation syndrome: proposed diagnostic criteria.J Allergy Clin Immunol. 2010; 126: 1099-1104Abstract Full Text Full Text PDF PubMed Scopus (225) Google Scholar, 22Valent P. Horny H.P. Triggiani M. Arock M. Clinical and laboratory parameters of mast cell activation as basis for the formulation of diagnostic criteria.Int Arch Allergy Immunol. 2011; 156: 119-127Crossref PubMed Scopus (39) Google Scholar, 23Hamilton M.J. Hornick J.L. Akin C. Castells M.C. Greenberger N.J. Mast cell activation syndrome: a newly recognized disorder with systemic clinical manifestations.J Allergy Clin Immunol. 2011; 128: 147-152.e2Abstract Full Text Full Text PDF PubMed Scopus (98) Google Scholar, 24Valent P. Akin C. Arock M. Brockow K. Butterfield J.H. Carter M.C. et al.Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal.Int Arch Allergy Immunol. 2012; 157: 215-225Crossref PubMed Scopus (423) Google Scholar, 25Valent P. Mast cell activation syndromes: definition and classification.Allergy. 2013; 68: 417-424Crossref PubMed Scopus (90) Google Scholar, 26Valent P. Akin C. Bonadonna P. Hartmann K. Brockow K. Niedoszytko M. et al.Proposed diagnostic algorithm for patients with suspected mast cell activation syndrome.J Allergy Clin Immunol Pract. 2019; 7: 1125-11233.e1Abstract Full Text Full Text PDF PubMed Scopus (112) Google Scholar Although in most of these patients, anaphylaxis is diagnosed, there are also patients who suffer from MCAS and fulfill all MCAS criteria, but do not necessarily exhibit the classical clinical features of anaphylaxis.In the past 10 years, diagnostic criteria and a classification for MCAS have been established by an international (European Union/US-based) consensus group.21Akin C. Valent P. Metcalfe D.D. Mast cell activation syndrome: proposed diagnostic criteria.J Allergy Clin Immunol. 2010; 126: 1099-1104Abstract Full Text Full Text PDF PubMed Scopus (225) Google Scholar,22Valent P. Horny H.P. Triggiani M. Arock M. Clinical and laboratory parameters of mast cell activation as basis for the formulation of diagnostic criteria.Int Arch Allergy Immunol. 2011; 156: 119-127Crossref PubMed Scopus (39) Google Scholar,24Valent P. Akin C. Arock M. Brockow K. Butterfield J.H. Carter M.C. et al.Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal.Int Arch Allergy Immunol. 2012; 157: 215-225Crossref PubMed Scopus (423) Google Scholar In addition, a diagnostic algorithm for MCAS has been developed.26Valent P. Akin C. Bonadonna P. Hartmann K. Brockow K. Niedoszytko M. et al.Proposed diagnostic algorithm for patients with suspected mast cell activation syndrome.J Allergy Clin Immunol Pract. 2019; 7: 1125-11233.e1Abstract Full Text Full Text PDF PubMed Scopus (112) Google Scholar However, there is still debate about the use of the term MCAS in various groups of patients, and many questions remain. For example, many patients with suspected MCAS and signs of MCA do not fulfill MCAS criteria.27Valent P. Bonadonna P. Hartmann K. Broesby-Olsen S. Brockow K. Butterfield J.H. et al.Why the 20% + 2 tryptase formula is a diagnostic gold standard for severe systemic mast cell activation and mast cell activation syndrome.Int Arch Allergy Immunol. 2019; 180: 44-51Crossref PubMed Scopus (61) Google Scholar, 28Gülen T. Akin C. Bonadonna P. Siebenhaar F. Broesby-Olsen S. Brockow K. et al.Selecting the right criteria and proper classification to diagnose mast cell activation syndromes: a critical review.J Allergy Clin Immunol Pract. 2021; 9: 3918-3928Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar, 29Valent P. Akin C. Hartmann K. Alvarez-Twose I. Brockow K. Hermine O. et al.Refined diagnostic criteria and classification of mast cell disorders: a consensus proposal.HemaSphere. 2021; 5: e646Crossref PubMed Google Scholar In these patients, MCA may be suspected as the major clinical problem, but it is a significant challenge to prove with certainty that the clinical features and symptoms are indeed derived from MC-dependent reactions and mediator release. Some of these patients have MCA disorders (MCADs) or nonspecified MCA reactions or suspected MCA.27Valent P. Bonadonna P. Hartmann K. Broesby-Olsen S. Brockow K. Butterfield J.H. et al.Why the 20% + 2 tryptase formula is a diagnostic gold standard for severe systemic mast cell activation and mast cell activation syndrome.Int Arch Allergy Immunol. 2019; 180: 44-51Crossref PubMed Scopus (61) Google Scholar, 28Gülen T. Akin C. Bonadonna P. Siebenhaar F. Broesby-Olsen S. Brockow K. et al.Selecting the right criteria and proper classification to diagnose mast cell activation syndromes: a critical review.J Allergy Clin Immunol Pract. 2021; 9: 3918-3928Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar, 29Valent P. Akin C. Hartmann K. Alvarez-Twose I. Brockow K. Hermine O. et al.Refined diagnostic criteria and classification of mast cell disorders: a consensus proposal.HemaSphere. 2021; 5: e646Crossref PubMed Google Scholar In these patients, local MCA, less severe MCA, or MCA potentially involving only a limited set of mediators or only 1 organ system may be implicated, whereas other patients do not have an MCAD.More recently, International Classification of Diseases-10-Clinical Modification (ICD-10-CM) codes have been created in the United States for most of these conditions, but there are no features or criteria through which these conditions can be diagnosed with certainty, leaving the possibility of overdiagnosis of MCA, which may lead to inappropriate patient management. To address this unmet need, our consensus group has worked out a proposal for disease-related clinical features and laboratory-based results that may qualify as indication of MCA and may even serve as criteria of MCAD not meeting MCAS criteria. In addition, we propose a global classification of MCA-related conditions, including predisposing conditions and clinically overt MCAD that can further be divided into (1) confirmed MCAS (and MCAS variants) and (2) other MCADs not fulfilling (all) diagnostic criteria to establish an MCAS, in contrast to cases of suspected MCAS/MCAD that are ruled out by history and/or laboratory evaluation.28Gülen T. Akin C. Bonadonna P. Siebenhaar F. Broesby-Olsen S. Brockow K. et al.Selecting the right criteria and proper classification to diagnose mast cell activation syndromes: a critical review.J Allergy Clin Immunol Pract. 2021; 9: 3918-3928Abstract Full Text Full Text PDF PubMed Scopus (15) Google ScholarDiagnostic Criteria and Classification of MCASThe diagnosis of MCAS is suspected when (1) clinical symptoms are severe, (2) systemic, involving at least 2 organ systems, and (3) recurrent, most commonly in the form of repeated anaphylaxis, and (4) evidence of involvement of MCs is demonstrable.21Akin C. Valent P. Metcalfe D.D. Mast cell activation syndrome: proposed diagnostic criteria.J Allergy Clin Immunol. 2010; 126: 1099-1104Abstract Full Text Full Text PDF PubMed Scopus (225) Google Scholar,22Valent P. Horny H.P. Triggiani M. Arock M. Clinical and laboratory parameters of mast cell activation as basis for the formulation of diagnostic criteria.Int Arch Allergy Immunol. 2011; 156: 119-127Crossref PubMed Scopus (39) Google Scholar,24Valent P. Akin C. Arock M. Brockow K. Butterfield J.H. Carter M.C. et al.Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal.Int Arch Allergy Immunol. 2012; 157: 215-225Crossref PubMed Scopus (423) Google Scholar, 25Valent P. Mast cell activation syndromes: definition and classification.Allergy. 2013; 68: 417-424Crossref PubMed Scopus (90) Google Scholar, 26Valent P. Akin C. Bonadonna P. Hartmann K. Brockow K. Niedoszytko M. et al.Proposed diagnostic algorithm for patients with suspected mast cell activation syndrome.J Allergy Clin Immunol Pract. 2019; 7: 1125-11233.e1Abstract Full Text Full Text PDF PubMed Scopus (112) Google Scholar, 27Valent P. Bonadonna P. Hartmann K. Broesby-Olsen S. Brockow K. Butterfield J.H. et al.Why the 20% + 2 tryptase formula is a diagnostic gold standard for severe systemic mast cell activation and mast cell activation syndrome.Int Arch Allergy Immunol. 2019; 180: 44-51Crossref PubMed Scopus (61) Google Scholar, 28Gülen T. Akin C. Bonadonna P. Siebenhaar F. Broesby-Olsen S. Brockow K. et al.Selecting the right criteria and proper classification to diagnose mast cell activation syndromes: a critical review.J Allergy Clin Immunol Pract. 2021; 9: 3918-3928Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar, 29Valent P. Akin C. Hartmann K. Alvarez-Twose I. Brockow K. Hermine O. et al.Refined diagnostic criteria and classification of mast cell disorders: a consensus proposal.HemaSphere. 2021; 5: e646Crossref PubMed Google Scholar Based on consensus criteria (Table I), the diagnosis of MCAS can be established when (a) typical clinical symptoms arising from recurrent acute systemic (multiorgan) MCA (typically in the form of recurrent anaphylaxis) have been documented, (b) MC-derived mediators increase substantially in serum or urine over the individual’s baseline (standard marker: documented increase in serum tryptase levels following the 120% + 2 ng/mL formula; eg, increase in serum tryptase from a basal level of 50 to 70: 50 + 10 [20%] = 60 + 2 = 62: any value beyond 62 counts as biochemical MCAS criterion), and (c) the symptoms respond to drugs blocking MCA, MC mediators, mediator production, or mediator effects.21Akin C. Valent P. Metcalfe D.D. Mast cell activation syndrome: proposed diagnostic criteria.J Allergy Clin Immunol. 2010; 126: 1099-1104Abstract Full Text Full Text PDF PubMed Scopus (225) Google Scholar,24Valent P. Akin C. Arock M. Brockow K. Butterfield J.H. Carter M.C. et al.Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal.Int Arch Allergy Immunol. 2012; 157: 215-225Crossref PubMed Scopus (423) Google Scholar, 25Valent P. Mast cell activation syndromes: definition and classification.Allergy. 2013; 68: 417-424Crossref PubMed Scopus (90) Google Scholar, 26Valent P. Akin C. Bonadonna P. Hartmann K. Brockow K. Niedoszytko M. et al.Proposed diagnostic algorithm for patients with suspected mast cell acti" @default.
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• W4281485518 date "2022-08-01" @default.
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• W4281485518 title "Global Classification of Mast Cell Activation Disorders: An ICD-10-CM–Adjusted Proposal of the ECNM-AIM Consortium" @default.
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