Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal
Open accessAbstract
Activation of tissue mast cells (MCs) and their abnormal growth and accumulation in various organs are typically found in primary MC disorders also referred to as mastocytosis. However, increasing numbers of patients are now being informed that their clinical findings are due to MC activation (MCA) that is neither associated with mastocytosis nor with a defined allergic or inflammatory reaction. In other patients with MCA, MCs appear to be clonal cells, but criteria for diagnosing mastocytosis are not met. A working conference was organized in 2010 with the aim to define criteria for diagnosing MCA and related disorders, and to propose a global unifying classification of all MC disorders and pathologic MC reactions. This classification includes three types of ‘MCA syndromes’ (MCASs), namely primary MCAS, secondary MCAS and idiopathic MCAS. MCA is now defined by robust and generally applicable criteria, including (1) typical clinical symptoms, (2) a substantial transient increase in serum total tryptase level or an increase in other MC-derived mediators, such as histamine or prostaglandin D 2, or their urinary metabolites, and (3) a response of clinical symptoms to agents that attenuate the production or activities of MC mediators. These criteria should assist in the identification and diagnosis of patients with MCAS, and in avoiding misdiagnoses or overinterpretation of clinical symptoms in daily practice. Moreover, the MCAS concept should stimulate research in order to identify and exploit new molecular mechanisms and therapeutic targets.
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Severe, episodic, and recurrent symptoms induced by MC mediators with concurrent involvement of at least two organ systems are required to meet the clinical consensus criterion of MCAS.
“Severe, episodic, and recurrent symptoms induced by MC mediators with concurrent involvement of at least two organ systems are required to meet the clinical consensus criterion of MCAS.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Clinical Symptoms section (p. 3) says symptoms affecting two or more organ systems increase the likelihood of systemic mast cell activation; it does not make that pattern a required clinical criterion. It also allows symptoms to be recurrent or permanent, contrary to the claim that episodic recurrence is required. The proposed criteria (p. 3) require typical symptoms alongside mediator and treatment-response evidence, not severe symptoms specifically. (LLM, conf=0.87)
Checked by a reasoning model — may be wrong.
Experimental — accuracy not yet measuredAccording to consensus criteria for MCAS diagnosis, sAT should be greater than sBT -1.2 + 2 ng/mL to confirm MCAS.
“According to the consensus criteria for the diagnosis of MCAS, sAT should be greater than sBT -1.2 þ 2 ng/mL to con fi rm the presence of MCAS.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The ‘Increase in Serum Tryptase Levels’ section (p. 4) sets the acute tryptase threshold at baseline plus 20% of baseline plus 2 ng/mL—not baseline minus 1.2 plus 2 ng/mL as written. The ‘Definition and Proposed MCA Criteria’ section (p. 3) also requires clinical and other evidence; the tryptase threshold alone does not confirm MCAS. (LLM, conf=0.87)
Checked by a reasoning model — may be wrong.
Experimental — accuracy not yet measuredMCAS is diagnosed when there is a documented clinically meaningful response to drugs that target mast cell mediators and/or suppress mast cell activation.
“a documented clinically meaningful response to drugs that either target MC-derived mediators (e.g., H1 antihistamines or antileukotrienes) and/or suppress MC activation (e.g., cromoglycate, ketotifen, or omalizumab) [19, 21, 22].”
From Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome (2019) · cites this paper as [19]
The Definition and Proposed MCA Criteria (p. 3) includes an objective major response to drugs affecting mast-cell mediators as one diagnostic criterion, and Response to Therapy (p. 4) names histamine receptor blockers, leukotriene receptor blockers and cromolyn. The paper requires clinical symptoms and a mediator increase as well; it does not establish a drug response alone as sufficient for diagnosis or name ketotifen or omalizumab among the examples. (LLM, conf=0.85)
Experimental — accuracy not yet measuredMCAS is diagnosed when there is documented recurrent episodic occurrence of typical systemic symptoms produced by mast cell mediators and involving at least two organ systems.
“documented recurrent episodic occurrence of typical systemic symptoms that are produced by MC mediators and involve at least 2 organ systems”
From Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome (2019) · cites this paper as [19]
The Clinical Symptoms section (p. 3) says recurrent symptoms involving two or more organ systems increase the likelihood of systemic mast cell activation. It also says symptoms alone are not diagnostic; the Definition and Proposed MCA Criteria section (p. 3) requires evidence of increased mast-cell mediators and generally a response to mediator-targeting treatment. The claim therefore describes a clinical criterion but overstates it as sufficient for an MCAS diagnosis. (LLM, conf=0.88)
Experimental — accuracy not yet measuredWell-recognized symptoms of systemic MCA include acute urticaria, flushing, abdominal cramping, diarrhea, hypotensive syncope or near syncope, and tachycardia.
“Well-recognized symptoms of systemic MCA include, among others, acute urticaria, flushing, abdominal cramping, diarrhea, hypotensive syncope or near syncope and tachycardia (Table 3) (1-3,26-28,31).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
The Clinical Symptoms section (p. 3) identifies acute urticaria, flushing, abdominal cramping, diarrhea and hypotension as suggestive of systemic mast cell activation. It does not identify hypotensive syncope or near syncope, or tachycardia, as symptoms; searches for those terms and related descriptions found no supporting passage. The paper also cautions that symptoms alone are not specific for systemic mast cell activation. (LLM, conf=0.86)
Experimental — accuracy not yet measured
Severe MCA fulfilling MCAS consensus criteria is almost always associated with acute severe recurrent symptoms affecting more than one organ or tissue, often with severe hypotension and anaphylaxis.
“severe MCA fulfilling MCAS consensus criteria is almost always associated with the occurrence of acute severe recurrent symptoms affecting more than one organ or tissue, often with severe hypotension and anaphylaxis (26-28).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
Clinical Symptoms (p. 3) says recurrent symptoms in two or more organ systems increase the likelihood of systemic MCA, and lists hypotension among suggestive symptoms. The Introduction (p. 2) describes a spectrum from mild symptoms to anaphylaxis and life-threatening hypotension, with symptoms that may be acute or chronic. The paper does not establish that MCAS meeting its criteria is almost always acute and severe, or that severe hypotension and anaphylaxis often occur. (LLM, conf=0.86)
Experimental — accuracy not yet measuredThe likelihood of MCA increases when two or more recognized symptoms are documented, and increases further when symptoms respond to agents blocking mediator effects, production, or secretion.
“The likelihood of MCA increases when two or more of such symptoms are documented, and the likelihood is even higher when the symptoms respond to agents blocking mediator effects, mediator production or mediator secretion.”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
The Clinical Symptoms section (p. 3) says the likelihood of systemic MCA increases when signs or symptoms occur in two or more organ systems in parallel, require therapy, and are recurrent or permanent—not merely when two symptoms are documented. The Response to Therapy section (p. 4) supports the second part: a major response to antimediator agents is highly suggestive of MCA, although responses to some agents are nonspecific. (LLM, conf=0.88)
Experimental — accuracy not yet measuredMCAS is classified into primary MCAS with clonal KIT-mutated CD25+ MC, secondary MCAS with an underlying non-neoplastic disease usually IgE-dependent allergy or hypersensitivity, and idiopathic MCAS with no KIT-mutated MC, no overt inflammatory disorder, and no hypersensitivity trigger.
“Based on the underlying condition, patients with MCAS should then be further classified into i) primary MCAS where KIT-mutated, clonal (CD25+) MC are detected (with or without an underlying diagnosis of mastocytosis), ii) secondary MCAS where an underlying non-neoplastic disease, usually an IgE-dependent allergy or other hypersensitivity reaction is detected, and iii) idiopathic MCAS, where no KIT”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
Split into 3 atoms — 2 confirmed, 1 partially supported, 0 disputed, 0 misattributed, 0 unverifiable. Expand the claim to see per-atom verdicts.
Experimental — accuracy not yet measuredIn cases with mastocytosis and MCA not fulfilling MCAS criteria, the consensus group recommends the diagnostic label SY for any MCA requiring continuous mediator-targeted therapy.
“The consensus group has recommended that in cases with mastocytosis (irrespective of the variant) any form of MCA requiring continuous mediator-targeted therapy should be marked by the diagnostic label ‘SY’”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
The Mastocytosis section (p. 7) says MCA can occur in any variant of mastocytosis and that its presence may be recorded with the subscript ‘SY’. The Response to Therapy section (p. 4) describes SM SY as mediator-related symptoms requiring therapy, but does not specify continuous therapy or explicitly recommend SY for cases that do not fulfill MCAS criteria. (LLM, conf=0.84)
Experimental — accuracy not yet measuredIf an IgE-dependent allergy or other underlying reactive disease is present without clonal MC, the diagnosis is secondary MCAS.
“If an IgE-dependent allergy or other underlying reactive disease (e.g., IgE-dependent or other hypersensitivity disorder) is present in the absence of clonal MC, the diagnosis is secondary MCAS (Table 2, Figure 2).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
The Classification of MCAS (pp. 5–6) identifies allergy or another underlying disease causing mast-cell activation as secondary MCAS, rather than primary clonal MCAS. But Table 4 also requires the criteria for mast-cell activation to be fulfilled; the presence of an allergy or reactive disease without clonal mast cells does not, by itself, establish secondary MCAS. (LLM, conf=0.82)
Experimental — accuracy not yet measuredSerum tryptase levels serve as the most specific marker and gold standard for measuring MC burden (sBT) as well as MCA and thus MCAS (sAT over sBT).
“Serum tryptase levels serve as the most speci fi c marker and gold standard for measuring the MC burden (sBT) as well as MCA and thus MCAS (sAT over sBT).”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
In “Increase in Serum Tryptase Levels” (p. 4), the paper calls tryptase the preferred marker for documenting mast-cell activation and specifies an acute rise of at least 20% plus 2 ng/ml over baseline as an MCAS criterion. It does not call tryptase the gold standard for measuring mast-cell burden; “Definition and Proposed MCA Criteria” (p. 3) also requires clinical criteria, so the tryptase rise alone does not establish MCAS. (LLM, conf=0.86)
Experimental — accuracy not yet measuredAnother required criterion of MCAS is a documented therapeutic response to specific drugs that target MC-derived mediators or mediator effects and/or suppress MCA.
“Another required criterion of MCAS is a documented therapeutic response to speci fi c drugs that target MC-derived mediators or mediator effects and/or suppress MCA”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Definition and Proposed MCA Criteria (p. 3) list an objective response to drugs that attenuate mast-cell mediators among the three usual criteria for systemic MCA. But the same passage allows MCA to be considered without a drug response in specified circumstances, so calling the response unconditionally required overstates the proposal. The Classification of MCAS (p. 6) uses fulfillment of MCA criteria to classify MCAS. (LLM, conf=0.82)
Experimental — accuracy not yet measuredThe 2012 MCAS proposal by Valent et al. recommends that an increase in BST levels of more than "20% + 2" (measured within 4 h from symptom onset) should be considered highly suggestive of MCAS.
“The 2012 proposal for mast cell activation syndrome (MCAS) by Valent et al. (30) recommends that an increase in BST levels of more than “20% + 2” (measured within 4 h from symptom onset) should be considered highly suggestive of MCAS.”
From Defining “Normal” basal serum tryptase levels: a context-dependent approach to improve diagnostics in systemic mastocytosis (2025) · cites this paper as [30]
Table 2 (p. 3) specifies a serum total tryptase increase of at least 20% above baseline plus 2 ng/ml during or within 4 hours of symptoms. The Increase in Serum Tryptase Levels section (p. 4) says this is indicative of mast cell activation and meets one MCAS criterion, not that it alone is highly suggestive of MCAS; the proposed diagnosis also considers symptoms and treatment response. (LLM, conf=0.87)
Experimental — accuracy not yet measuredAn increasing number of patients are being given a misdiagnosis of MCAS based on nonspecific criteria.
“as an increasing number of patients are being given a misdiagnosis of MCAS based on nonspeci fi c criteria”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Abstract (p. 1) says increasing numbers of patients are being told their findings are due to mast-cell activation. The Impact of the Classification on Research and Practice (p. 8) says patients with poorly defined symptoms are sometimes diagnosed without a solid diagnostic basis. The paper does not say that the number of MCAS misdiagnoses is increasing. (LLM, conf=0.87)
Experimental — accuracy not yet measuredMany patients referred with suspected MCAS fail to respond to MCAS therapies.
“and thus fail to respond to MCAS therapies.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Definition and Proposed MCA Criteria section (p. 3) says that, in some circumstances, a patient may not respond to a drug. It does not say that many patients referred with suspected MCAS fail to respond to MCAS therapies, so the claimed frequency and referral population are unsupported. (LLM, conf=0.85)
Experimental — accuracy not yet measuredThe definition of MCAS after the Vienna consensus is based on three diagnostic criteria that must be fulfilled before diagnosis: typical MCA-related clinical symptoms; an event-related increase in serum tryptase above baseline according to the formula sBT + 20% of sBT plus 2 ng/mL; and response to drugs directed against MCA or mediator production/effects.
“The de fi nition of MCAS after the Vienna consensus is based on three diagnostic criteria that have to be ful fi lled before the diagnosis of MCAS can be established: (1) typical (MCA-related) clinical symptoms; (2) an event-related increase in serum tryptase above the individual ' s baseline tryptase (sBT), according to the formula: sBT þ 20% of sBT ( ¼ 120% of sBT ¼ sBT -1.2) plus 2 ng/mL; and (”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
Split into 2 atoms — 1 confirmed, 1 partially supported, 0 disputed, 0 misattributed, 0 unverifiable. Expand the claim to see per-atom verdicts.
Experimental — accuracy not yet measuredAn increasing number of patients who believe they are experiencing MCAS are being referred to specialized centers.
“Thus, an increasing number of patients who believe that they are, or have been informed that they are, experiencing MCAS are being referred to specialized centers.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Abstract (p. 1) says increasing numbers of patients are being informed that their findings are due to mast-cell activation, supporting the claim's trend in perceived diagnoses. But the section on patient concerns (p. 9) says patients want more referral centers; it does not say that referrals to specialized centers are increasing. (LLM, conf=0.84)
Experimental — accuracy not yet measuredMany patients referred with suspected MCAS have an unrelated disease.
“In many instances, these patients have an unrelated disease”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Introduction (p. 2) says that some patients have symptoms mimicking mast-cell activation without mast-cell involvement, and Differential Diagnoses (p. 8) lists common conditions that can be confused with it. The paper does not establish that many patients referred with suspected MCAS have such an unrelated disease. (LLM, conf=0.82)
Experimental — accuracy not yet measuredMany patients referred with suspected MCAS do not fulfill the classical stringent Vienna consensus criteria of MCAS.
“do not ful fi ll the classical stringent Vienna consensus criteria of MCAS”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Definition and Proposed MCA Criteria section (p. 3) sets out diagnostic criteria, and the Classification of MCAS section (p. 6) requires MCA criteria to be fulfilled for MCAS. The paper does not report that many patients referred with suspected MCAS fail to meet those criteria. (LLM, conf=0.84)
Experimental — accuracy not yet measuredThe most frequently reported symptoms of MCAS encompass cardiovascular, cutaneous, gastrointestinal, respiratory, and naso-ocular symptoms.
“The most frequently reported symptoms of MCAS encompass cardiovascular, cutaneous, gastrointestinal, respiratory, and naso-ocular symptoms.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
Table 3 (Definition and Proposed MCA Criteria, p. 3) lists typical symptoms spanning the claimed systems, including hypotension, flushing, diarrhea, wheezing, and nasal congestion. The Clinical Symptoms section (p. 3) likewise describes symptoms across multiple organ systems. Neither passage establishes that these are the most frequently reported symptoms of MCAS; Table 3’s percentages indicate expert consensus, not patient-reported frequency. (LLM, conf=0.86)
Experimental — accuracy not yet measuredSome patients may easily be given an incorrect diagnosis of MCAS when less stringent criteria are applied, but not when validated Vienna consensus criteria are used.
“Some of these patients may easily be given the incorrect diagnosis of having MCAS when applying less stringent criteria, but not the validated Vienna consensus criteria. 28,30”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Impact of the Classification on Research and Practice (p. 8) says patients with poorly defined symptoms are sometimes diagnosed without a solid basis and that the proposed criteria may help avoid misdiagnosis. The Concluding Remarks (p. 9) likewise say routine use should be helpful, but the paper presents the criteria as a proposal; it does not establish that they were validated or that using them rules out an incorrect MCAS diagnosis. (LLM, conf=0.86)
Experimental — accuracy not yet measuredThe sensitivity of the tryptase algorithm decreases with decreasing clinical severity and with delayed blood draws after symptom resolution.
“However, the sensitivity of the tryptase algorithm decreases with decreasing clinical severity and with delayed blood draws after resolution of clinical symptoms.”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
Response to mediator-blocking drugs is a criterion of MCAS.
“Indeed, the response to such drugs is helpful in practice and is therefore a criterion of MCAS (26-28).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
A minimal increase of acute serum tryptase to greater than 20% of baseline plus 2 ng/mL absolute strongly supports the diagnosis of MCAS.
“a minimal increase of the acute serum tryptase level to greater than plus 20% of baseline plus 2 ng/mL absolute tryptase strongly supports the diagnosis of MCAS (27).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
Tryptase is more specific for MC than other mediators and is considered the most precise parameter for demonstrating and documenting MCA.
“Some mediators, like tryptase, are more specific than others for MC and thereby considered as the most precise parameters for the demonstration and documentation of MCA (27,28,37-40).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
A rapid substantial increase in serum tryptase above the individual baseline is considered specific for MC involvement and MCA and can be used as an MCAS criterion.
“Therefore, in a routine evaluation, a rapid substantial increase in serum tryptase levels above the individual’s baseline is considered specific for MC involvement and MCA, and thus can be employed as a MCAS criterion.”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
If a major increase in PGD2 metabolite is found and symptoms respond to cyclooxygenase inhibitors, MCAS may be considered, although the cell source may be ambiguous if tryptase and histamine or histamine metabolite levels are normal.
“Whenever a major increase in the PGD2 metabolite level is found and the symptoms respond to cyclooxygenase inhibitors, the diagnosis of MCAS may also be considered (27) although the cell source may be ambiguous, particularly if both tryptase and histamine or histamine metabolite levels are normal.”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
Diagnostic MCAS criteria include typical clinical symptoms often with hypotension, an event-related substantial increase in serum tryptase, and symptom response to MC-stabilizing drugs or drugs counteracting MC mediators.
“Diagnostic MCAS criteria include typical clinical symptoms, often with hypotension, an event-related, substantial increase in serum tryptase levels and response of clinical symptoms to MC-stabilizing drugs or drugs counteracting the effects of MC-derived mediators.”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
If no evidence for primary or secondary MCAS is found, the patient is classified as having idiopathic MCAS.
“If no evidence for primary or secondary MCAS is found, the patient is classified as having idiopathic MCAS (Table 2, Figure 1) (26-28).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
Experimental — accuracy not yet measuredPatients with clonal KIT-mutated CD25+ MC but only one or two minor SM criteria and no major SM criterion, as well as those with cutaneous mastocytosis or SM, are classified as primary clonal MCAS.
“These patients as well as those with cutaneous mastocytosis (CM) or SM are both classified as primary (clonal) MCAS (Table 2).”
From Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome (2019) · cites this paper as [27]
There are MCAS patients in whom no KIT-mutated mast cells, no allergy, and no overt inflammatory disorders or triggers of a hypersensitivity reaction are detected; these patients have idiopathic MCAS.
“Finally, there are MCAS patients in whom no KIT -mutated MCs, no allergy, and no overt in fl ammatory disorders or triggers of a hypersensitivity reaction are detected. These patients have idiopathic MCAS. 28,30”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
Nine publications offering new or modified schemes for diagnosing the full range of MCAS were found, and each fell into one of two principal schools of thought.
“We found nine such publications [7][8][9][19][20][21][22][23][24], reviewed them, and found each to fall into one of two principal schools of thought as defined by their original papers [8,9].”
From Diagnosis of mast cell activation syndrome: a global "consensus-2" (2021) · cites this paper as [9]
The Vienna consensus group concluded that MCAS is a heterogeneous disorder.
“This consensus group concluded that MCAS is a heterogeneous disorder”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The acute serum tryptase level, when significantly elevated from the individual's baseline, is a specific marker for MCA and thus MCAS, although sensitivity varies with the severity of the reaction and timing of collection.
“The acute serum tryptase (sAT) level, when signi fi cantly elevated from the individual ' s baseline, is a speci fi c marker for MCA and thus MCAS, although sensitivity varies with the severity of the reaction and timing of collection.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Vienna consensus requires clinical symptoms of MCAS to be associated with an acute, event-related increase in the levels of a validated marker of systemic MCA.
“The Vienna consensus requires clinical symptoms of MCAS to be associated with an acute, event-related increase in the levels of a validated marker of systemic MCA.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
The Vienna consensus group concluded that MCAS diagnosis should be rendered only when specific diagnostic criteria for MCAS are met.
“and that its diagnosis should be rendered only when speci fi c diagnostic criteria for MCAS are met.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
Primary MCAS includes conditions characterized by MCAS criteria and a population of clonal mast cells exhibiting an activating mutation in KIT, usually KIT D816V, and/or aberrant expression of CD25.
“Primary MCAS includes conditions characterized by MCAS criteria and a population of clonal MCs exhibiting an activating mutation in KIT , usually KIT D816V, and/or aberrant expression of CD25. 28”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
Based on the Vienna consensus, MCAS can be divided into primary (clonal) MCAS, secondary (nonclonal) MCAS, and idiopathic MCAS.
“Based on the Vienna consensus, MCAS can be divided into primary (clonal) MCAS, secondary (nonclonal) MCAS, and idiopathic MCAS (Figure 2). 28,30”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [28]
Lifetime risk of anaphylaxis in mastocytosis is significantly increased in adults, approximately 49%.
“Lifetime risk of anaphylaxis in mastocytosis is significantly increased in adults (approximately 49%). 120,121”
From Sinus Tachycardia: a Multidisciplinary Expert Focused Review (2022) · cites this paper as [121]
A formula of acute event-related tryptase increase of 20% from baseline plus 2 ng/ml has been proposed as a clinically significant confirmation of mast cell activation.
“A formula of acute event related tryptase increase of 20% from baseline level +2 ng/ml has been proposed as a clinically significant confirmation of mast cell activation121,122.”
From Sinus Tachycardia: a Multidisciplinary Expert Focused Review (2022) · cites this paper as [121]
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“LABORATORY ASSESSMENTS IN PATIENTS WITH SUSPECTED MCA MCA is associated with the release of preformed and newly generated mediators and their effects on various target cells.(26-28,31) In severe systemic reactions, increased levels of MCderived mediators should be measurable in biological fluids.”
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- Authors
- Valent, P., Akin, C., Arock, M., Brockow, K., Butterfield, J. H., Carter, M. C., Castells, M., Escribano, L., Hartmann, K., Lieberman, P., Nedoszytko, B., Orfao, A., Schwartz, L. B., Sotlar, K., Sperr, W. R., Triggiani, M., Valenta, R., Horny, H.-P., & Metcalfe, D. D.
- Affiliations
- Medical University of Vienna (AT)
- Brigham and Women's Hospital (US)
- École Normale Supérieure Paris-Saclay (FR)
- Technical University of Munich (DE)
- Mayo Clinic (US)
- National Institutes of Health (US)
- University of Cologne (DE)
- University of Tennessee System (US)
- Virginia Commonwealth University (US)
- Pathologisches Institut (DE)
- Federico II University Hospital (IT)
- ORCID
- Venue
- International Archives of Allergy and Immunology
- Publisher
- S. Karger AG
- Published
- 2011-10-27
- Volume
- 157
- Issue
- 3
- Pages
- 215-225
- ISSN
- 1018-2438 (ISSN-L)
- 1423-0097
- PMC
- PMC3224511
- OpenAlex
- W2139695498
- Cited by
- 654
- References
- 61
- Citations by year
- 2026: 34 · 2025: 35 · 2024: 44 · 2023: 63 · 2022: 49 · 2021: 61 · 2020: 51 · 2019: 54 · 2018: 40 · 2017: 42 · 2016: 34 · 2015: 52 · 2014: 45 · 2013: 33 · 2012: 15
- FWCI
- 13.33
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- International Archives of Allergy and Immunology (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- edoc (University of Basel) (submittedVersion)
- Open access LMU (Ludwid Maxmilian's Universitat Munchen) (submittedVersion)
- mediaTUM (Technical University of Munich) (submittedVersion)
- Funders
- National Institutes of Health
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Physiology › Asthma and respiratory diseases
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal motility and disorders
- Keywords
- mast cell activation syndrome, mast cell disease, mastocytosis, mast cell activation, diagnostic criteria, classification, tryptase, histamine, prostaglandin D2, mast cell mediators
- MeSH terms
- Attitude to Health, Diagnosis, Differential, Humans, Mast Cells, Mastocytosis, Biomarkers, Tryptases
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