Mast cell activation syndromes: definition and classification
Open accessAbstract
Mast cell activation (MCA) occurs in a number of different clinical conditions, including I g E‐dependent allergies, other inflammatory reactions, and mastocytosis. MCA‐related symptoms may be mild, moderate, severe, or even life‐threatening. The severity of MCA depends on a number of different factors, including genetic predisposition, the number and releasability of mast cells involved in the reaction, the type of allergen, presence of specific I g E, and presence of certain comorbidities. In severe reactions, MCA can be documented by a substantial increase in the serum tryptase level above baseline. When symptoms are recurrent, are accompanied by an increase in mast cell–derived mediators in biological fluids, and are responsive to treatment with mast cell–stabilizing or mediator‐targeting drugs, the diagnosis of mast cell activation syndrome (MCAS) is appropriate. Based on the underlying condition, these patients can further be classified into i) primary MCAS where KIT‐mutated, clonal mast cells are detected, ii) secondary MCAS where an underlying inflammatory disease, often in the form of an I g E‐dependent allergy, but no KIT‐mutated mast cells, is found, and iii) idiopathic MCAS, where neither an allergy or other underlying disease, nor KIT‐mutated mast cells are detectable. It is important to note that in many patients with MCAS, several different factors act together to lead to severe or even life‐threatening anaphylaxis. Detailed knowledge about the pathogenesis and complexity of MCAS, and thus establishing the exact final diagnosis, may greatly help in the management and therapy of these patients.
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Well-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 [28]
The Symptoms of mast cell activation section and its Table 1 list acute urticaria, flushing, abdominal cramping, diarrhea, hypotension, and tachycardia as MCA-related findings. They do not identify syncope or near syncope as MCA symptoms; the paper mentions syncope only in a differential diagnosis. The claim is therefore supported except for that specific description of hypotension. (LLM, conf=0.83)
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 [28]
The Laboratory assessment of systemic MCA section says mast-cell mediators are measurable in most severe systemic reactions and describes a rapid rise in tryptase from baseline as a diagnostic parameter. It recommends measuring the baseline after symptoms resolve, but does not state that the algorithm’s sensitivity decreases progressively with milder symptoms or quantify the effect of delayed acute blood draws. The Differential diagnoses section likewise calls for a post-resolution sample to establish baseline, not as a substitute for an acute sample. (LLM, conf=0.77)
Experimental — accuracy not yet measuredSevere 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 [28]
The Definition of MCA syndromes section describes MCAS as severe recurrent or chronic systemic mast-cell activation, and the Symptoms section says isolated symptoms are not typical. Table 1 identifies hypotension or shock as a key finding, and the Symptoms section describes anaphylaxis as a possible severe manifestation. The claim’s emphasis on almost-always acute, recurrent episodes goes beyond the criteria, which expressly allow chronic symptoms; the copy also does not establish how often anaphylaxis accompanies MCAS. (LLM, conf=0.82)
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 [28]
The ‘Underlying disorders and classification of MCAS’ section classifies MCAS with KIT-mutated, usually CD25-positive clonal mast cells as primary, including cases with CM or SM and cases with neither. Our copy does not specify the claimed threshold of one or two minor SM criteria and no major criterion. (LLM, conf=0.85)
Experimental — accuracy not yet measuredIn patients with clonal MC, IgE-dependent and IgE-independent MCA reactions may be severe, often in the form of anaphylaxis, depending on inherent hyperreactivity of MCs and the disease context.
“In patients with clonal MC, IgE-dependent and IgE-independent MCA reactions may be severe (often in the form of anaphylaxis), depending on both the inherent hyperreactivity of MCs and the disease context”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [16]
The Abstract (page unavailable) identifies clonal mast cells in primary MCAS and says MCA can be severe or life-threatening, with severity depending on mast-cell releasability and other factors. “The impact of comorbidities in patients with MCAS” (page unavailable) describes severe anaphylaxis when mastocytosis coexists with IgE-dependent allergy. Our copy does not specifically establish that IgE-independent reactions in patients with clonal mast cells can likewise be severe. (LLM, conf=0.80)
Experimental — accuracy not yet measured
In patients with secondary MCAS, mast cells are normal (nonclonal) cells even if they are sometimes slightly elevated in tissue sections (reactive).
“In patients with secondary MCAS, MCs are normal (nonclonal) cells even if they are sometimes slightly elevated in tissue sections (reactive). 16,97”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [16]
The classification section distinguishes secondary MCAS from primary MCAS with clonal mast cells, and Table 3 says a normal, CD25-negative mast-cell phenotype supports secondary MCAS. Our copy does not state that mast cells in secondary MCAS are sometimes slightly elevated in tissue sections or describe such an increase as reactive. (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 [28]
MCAS 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 [28]
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 [28]
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 [28]
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 [28]
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 [28]
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 [28]
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 [28]
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Holgate, S. T., Robinson, C., & Church, M. K. (1988) · Allergy · doi:10.1111/j.1398-9995.1988.tb05044.x · Cited by 16 · see citing works
Salicylamide‐induced anaphylaxis: increased urinary leukotriene E4 and prostaglandin D2 metabolite
Ono, E., Taniguchi, M., Mita, H., & Akiyama, K. (2008) · Allergy · doi:10.1111/j.1398-9995.2007.01616.x · Cited by 9 · see citing works
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Sorted by citations
The diagnosis and management of acute and chronic urticaria: 2014 update
Bernstein, J. A., Lang, D. M., Khan, D. A., Craig, T., Dreyfus, D. H., Hsieh, F. H., Sheikh, J. K., Weldon, D., Zuraw, B. L., Bernstein, D. I., et al. (2014) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2014.02.036 · Cited by 685 · see citing works
“Finally, mast cell activation syndromes can present with episodic urticaria or angioedema, typically along with other systemic symptoms, including gastrointestinal, respiratory, or cardiovascular symptoms.(423) Summary Statement 66: EM can resemble urticaria and might be caused by viral infections (eg, herpes), mycopl…”
BackgroundUpdated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal
Valent, P., Akin, C., Hartmann, K., Alvarez-Twose, I., Brockow, K., Hermine, O., Niedoszytko, M., Schwaab, J., Lyons, J. J., Carter, M. C., et al. (2021) · HemaSphere · doi:10.1097/hs9.0000000000000646 · Cited by 317 · see citing works
Self-citationWorld Allergy Organization Anaphylaxis Guidelines: 2013 update of the evidence base
Simons, F. E. R., Ardusso, L. R. F., Dimov, V., Ebisawa, M., El-Gamal, Y. M., Lockey, R. F., Sanchez-Borges, M., Senna, G. E., Sheikh, A., Thong, B. Y., et al. (2013) · International Archives of Allergy and Immunology · doi:10.1159/000354543 · Cited by 269 · see citing works
Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome
Valent, P., Akin, C., Bonadonna, P., Hartmann, K., Brockow, K., Niedoszytko, M., Nedoszytko, B., Siebenhaar, F., Sperr, W. R., Oude Elberink, J. N. G., et al. (2019) · The Journal of Allergy and Clinical Immunology: In Practice · doi:10.1016/j.jaip.2019.01.006 · Cited by 220 · see citing works
“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.”
BackgroundMethodInfluentialSelf-citationValent, P., Escribano, L., Broesby‐Olsen, S., Hartmann, K., Grattan, C., Brockow, K., Niedoszytko, M., Nedoszytko, B., Oude Elberink, J. N. G., Kristensen, T., et al. (2014) · Allergy · doi:10.1111/all.12436 · Cited by 174 · see citing works
Self-citationHuman mast cell tryptase in biology and medicine
Vitte, J. (2015) · Molecular Immunology · doi:10.1016/j.molimm.2014.04.001 · Cited by 152 · see citing works
Valent, P., Bonadonna, P., Hartmann, K., Broesby-Olsen, S., Brockow, K., Butterfield, J. H., Triggiani, M., Lyons, J. J., Oude Elberink, J. N. G., Arock, M., et al. (2019) · International Archives of Allergy and Immunology · doi:10.1159/000501079 · Cited by 146 · see citing works
Self-citationPicard, M., Özyiğit, L. P., Caiado, J., Junior, P. F. G.-B., Galvão, V. R., Berlin, S. T., Campos, S. M., Matulonis, U. A., & Castells, M. (2015) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2015.10.039 · Cited by 128 · see citing works
Clinical development methodology for infusion‐related reactions with monoclonal antibodies
Doessegger, L., & Banholzer, M. L. (2015) · Clinical & Translational Immunology · doi:10.1038/cti.2015.14 · Cited by 127 · see citing works
Kucharik, A. H., & Chang, C. (2020) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-019-08755-8 · Cited by 122 · see citing works
Drug hypersensitivity in clonal mast cell disorders: ENDA / EAACI position paper
Bonadonna, P., Pagani, M., Aberer, W., Bilò, M. B., Brockow, K., Elberink, H. O., Garvey, L. H., Mosbech, H., Romano, A. G., Zanotti, R., et al. (2015) · Allergy · doi:10.1111/all.12617 · Cited by 102 · see citing works
Carter, M. C., Desai, A., Komarow, H. D., Bai, Y., Clayton, S. T., Clark, A. S., Ruiz-Esteves, K. N., Long, L. M., Cantave, D., Wilson, T. M., et al. (2018) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2017.05.036 · Cited by 97 · see citing works
Dengue virus–elicited tryptase induces endothelial permeability and shock
Rathore, A. P. S., Mantri, C. K., Aman, S. A. B., Syenina, A., Ooi, J. S. G., Jagaraj, C. J., Goh, C. C., Tissera, H. A., Wilder‐Smith, A., Ng, L. G., et al. (2019) · Journal of Clinical Investigation · doi:10.1172/jci128426 · Cited by 94 · see citing works
Valent, P., Akin, C., Nedoszytko, B., Bonadonna, P., Hartmann, K., Niedoszytko, M., Brockow, K., Siebenhaar, F., Triggiani, M., Arock, M., et al. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21239030 · Cited by 92 · see citing works
Self-citationValent, P., Hartmann, K., Bonadonna, P., Gülen, T., Brockow, K., Alvarez-Twose, I., Hermine, O., Niedoszytko, M., Carter, M. C., Hoermann, G., et al. (2022) · The Journal of Allergy and Clinical Immunology: In Practice · doi:10.1016/j.jaip.2022.05.007 · Cited by 90 · see citing works
Self-citationClinical characteristics and risk profile of patients with elevated baseline serum tryptase
Fellinger, C., Hemmer, W., Wöhrl, S., Sesztak‐Greinecker, G., Jarisch, R., & Wantke, F. (2014) · Allergologia et Immunopathologia · doi:10.1016/j.aller.2014.05.002 · Cited by 89 · see citing works
Familial Occurrence of Systemic Mast Cell Activation Disease
Molderings, G. J., Haenisch, B., Bogdanow, M., Fimmers, R., & Nöthen, M. M. (2013) · PLoS ONE · doi:10.1371/journal.pone.0076241 · Cited by 94 · see citing works
“In the three multiply affected pedigrees in which we characterized somatic KIT mutations and expression of KIT isoforms, severity of the MCAD was defined as recently proposed (mild: no drug therapy required; moderate: drug therapy required, but no need for hospitalization; severe: drug therapy and recurrent hospitaliz…”
BackgroundNew Insights into the Pathogenesis of Mastocytosis: Emerging Concepts in Diagnosis and Therapy
Valent, P., Akin, C., Sperr, W. R., Horny, H., Arock, M., Metcalfe, D. D., & Galli, S. J. (2022) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathmechdis-031521-042618 · Cited by 87 · see citing works
Self-citationGülen, T., Akin, C., Bonadonna, P., Siebenhaar, F., Broesby-Olsen, S., Brockow, K., Niedoszytko, M., Nedoszytko, B., Oude Elberink, H. N. G., Butterfield, J. H., et al. (2021) · The Journal of Allergy and Clinical Immunology: In Practice · doi:10.1016/j.jaip.2021.06.011 · Cited by 83 · see citing works
“In patients with secondary MCAS, MCs are normal (nonclonal) cells even if they are sometimes slightly elevated in tissue sections (reactive).(16,97) These patients usually have an IgE-dependent allergy or another hypersensitivity disorder.”
BackgroundSelf-citationValent, P., Akin, C., Gleixner, K. V., Sperr, W. R., Reiter, A., Arock, M., & Triggiani, M. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20122976 · Cited by 84 · see citing works
Self-citationGülen, T., Hägglund, H., Dahlén, B., & Nilsson, G. (2016) · Journal of Internal Medicine · doi:10.1111/joim.12410 · Cited by 75 · see citing works
Histamine intolerance and dietary management: A complete review
Martín, I. S. M., Brachero, S., & Vilar, E. G. (2016) · Allergologia et Immunopathologia · doi:10.1016/j.aller.2016.04.015 · Cited by 77 · see citing works
Hymenoptera Allergy and Mast Cell Activation Syndromes
Bonadonna, P., Bonifacio, M., Lombardo, C., & Zanotti, R. (2015) · Current Allergy and Asthma Reports · doi:10.1007/s11882-015-0582-5 · Cited by 68 · see citing works
Mast Cells in Liver Fibrogenesis
Weiskirchen, R., Meurer, S. K., Liedtke, C., & Huber, M. (2019) · Cells · doi:10.3390/cells8111429 · Cited by 59 · see citing works
“Additionally, clinical conditions related to MC activation, where symptoms are recurrent, are accompanied by an increase in MC-derived mediators in biological fluids, and are responsive to treatment with MC-stabilizing or mediator-targeting drugs, can/might be diagnosed as MC activation syndrome (MCAS) [32] (for a pro…”
BackgroundMast cell activation syndromes: Collegium Internationale Allergologicum update 2022
Valent, P., Hartmann, K., Bonadonna, P., Niedoszytko, M., Triggiani, M., Arock, M., & Brockow, K. (2022) · International Archives of Allergy and Immunology · doi:10.1159/000524532 · Cited by 55 · see citing works
Self-citationValent, P., Elberink, J. N. G. O., Górska, A., Lange, M., Zanotti, R., van Anrooij, B., Bonifacio, M., Bonadonna, P., Gleixner, K. V., Hadzijusufovic, E., et al. (2018) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2018.09.024 · Cited by 56 · see citing works
Self-citationMastocytosis: a paradigmatic example of a rare disease with complex biology and pathology.
Valent, P. (2013) · Europe PMC (PubMed Central) · Cited by 55 · see citing works
Self-citationMessingham, K. N., Holahan, H. M., & Fairley, J. A. (2014) · Immunologic Research · doi:10.1007/s12026-014-8547-7 · Cited by 54 · see citing works
Boehm, T., Reiter, B., Ristl, R., Petroczi, K., Sperr, W., Stimpfl, T., Valent, P., & Jilma, B. (2019) · Allergy · doi:10.1111/all.13663 · Cited by 53 · see citing works
Self-citationFamilial hypertryptasemia with associated mast cell activation syndrome
Sabato, V., Van De Vijver, E., Hagendorens, M., Vrelust, I., Reyniers, E., Fransen, E., Bridts, C., De Clerck, L., Mortier, G., Valent, P., et al. (2014) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2014.06.007 · Cited by 52 · see citing works
Anaphylaxis as a clinical manifestation of clonal mast cell disorders
Matito, A., Alvarez-Twose, I., Morgado, J. M., Sánchez-Muñoz, L., Orfao, A., & Escribano, L. (2014) · Current Allergy and Asthma Reports · doi:10.1007/s11882-014-0450-8 · Cited by 51 · see citing works
Mobilization and Activation of the Innate Immune Response to Dengue Virus
King, C. A., Wegman, A. D., & Endy, T. P. (2020) · Frontiers in Cellular and Infection Microbiology · doi:10.3389/fcimb.2020.574417 · Cited by 50 · see citing works
“Mast cell activation [reviewed in (Valent, 2013; Afrin et al., 2017)] is known to mediate several of the common clinical symptoms associated with dengue patients including rash, diarrhea, vomiting, headache, eye pain/inflammation and muscle pain.”
BackgroundDoctor, I think I am suffering from MCAS: differential diagnosis and separating facts from fiction
Valent, P., & Akin, C. (2019) · The Journal of Allergy and Clinical Immunology: In Practice · doi:10.1016/j.jaip.2018.11.045 · Cited by 47 · see citing works
Self-citationOutcomes of Allergy/Immunology Follow-Up After an Emergency Department Evaluation for Anaphylaxis
Campbell, R. L., Park, M. A., Kueber, M. A., Lee, S., & Hagan, J. B. (2014) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2014.07.011 · Cited by 48 · see citing works
“Age (y), median (IQR) 34 (15-52) FAAN- F ood Allergy and Anaphylaxis Network”
BackgroundDiagnosis and management of mastocytosis: an emerging challenge in applied hematology
Valent, P. (2015) · Hematology · doi:10.1182/asheducation-2015.1.98 · Cited by 48 · see citing works
Self-citationValent, P. (2014) · Clinical & Experimental Allergy · doi:10.1111/cea.12318 · Cited by 47 · see citing works
Self-citationThe amelioration effect of tranexamic acid in wrinkles induced by skin dryness
Hiramoto, K., Sugiyama, D., Takahashi, Y., & Mafune, E. (2016) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2016.02.013 · Cited by 45 · see citing works
Butterfield, J., & Weiler, C. R. (2020) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2020.02.021 · Cited by 41 · see citing works
H1‐antihistamines for primary mast cell activation syndromes: a systematic review
Nurmatov, U. B., Rhatigan, E., Simons, F. E. R., & Sheikh, A. (2015) · Allergy · doi:10.1111/all.12672 · Cited by 39 · see citing works
“Mastocytosis experts have typically recommended oral (old) first-generation, sedating H1-antihistamines such as azelastine, chlorpheniramine, diphenhydramine, hydroxyzine, and ketotifen for the prevention and treatment of symptoms (1, 2, 4, 5).”
BackgroundClinical Impact of Inherited and Acquired Genetic Variants in Mastocytosis
Nedoszytko, B., Arock, M., Lyons, J. J., Bachelot, G., Schwartz, L. B., Reiter, A., Jawhar, M., Schwaab, J., Lange, M., Greiner, G., et al. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22010411 · Cited by 37 · see citing works
Self-citationChen, X., Xu, Z., Zeng, S., Wang, X., Liu, W., Qian, L., Wei, J., Yang, X., Shen, Q., Gong, Z., et al. (2019) · Frontiers in Oncology · doi:10.3389/fonc.2019.00852 · Cited by 36 · see citing works
World Health Organization Classification and Diagnosis of Mastocytosis
Valent, P., Sotlar, K., Horny, H., Arock, M., & Akin, C. (2023) · Immunology and Allergy Clinics of North America · doi:10.1016/j.iac.2023.04.011 · Cited by 36 · see citing works
Self-citationThe Role of Mast Cells in the Induction and Maintenance of Inflammation in Selected Skin Diseases
Woźniak, E., Owczarczyk-Saczonek, A. B., Lange, M., Czarny, J. H., Wygonowska, E., Placek, W. J., & Nedoszytko, B. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms24087021 · Cited by 35 · see citing works
“The symptoms respond to drugs blocking the activation of MCs, MC mediators, mediator production, or mediator effects [29,40,56,57].”
BackgroundMast cell tryptase – Marker and maker of cardiovascular diseases
Mohajeri, M., Kovanen, P. T., Bianconi, V., Pirro, M., Cicero, A. F. G., & Sahebkar, A. (2019) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2019.03.008 · Cited by 35 · see citing works
The Spectrum of Aggressive Mastocytosis: A Workshop Report and Literature Review
Leguit, R. J., Hebeda, K. M., Kremer, M., van der Walt, J. D., Gianelli, U., Tzankov, A., & Orazi, A. (2019) · Pathobiology · doi:10.1159/000504099 · Cited by 35 · see citing works
Feldweg, A. M. (2015) · Immunology and Allergy Clinics of North America · doi:10.1016/j.iac.2015.01.005 · Cited by 35 · see citing works
Schlapbach, C., & Simon, D. (2014) · Allergy · doi:10.1111/all.12529 · Cited by 34 · see citing works
“, increase in tryptase), and 3) the symptoms respond to therapy with mast cell-stabilizing agents or drugs directed against mast cell mediator production, mediator release, or mediator effects (85).”
BackgroundValent, P., Akin, C., Bonadonna, P., Brockow, K., Niedoszytko, M., Nedoszytko, B., Butterfield, J. H., Álvarez‐Twöse, I., Sotlar, K., Schwaab, J., et al. (2020) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2020.06.009 · Cited by 32 · see citing works
“In severe cases, MCAS may be diagnosed.(10-12) MCAS can also occur in the absence of mastocytosis.”
BackgroundInfluentialSelf-citationRussell, N., Jennings, S., Jennings, B., Slee, V., Sterling, L., Castells, M., Valent, P., & Akin, C. (2019) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2018.07.032 · Cited by 31 · see citing works
Self-citation(2023) · The Journal of Allergy and Clinical Immunology: In Practice · doi:10.1016/j.jaip.2023.07.001 · Cited by 25 · see citing works
Self-citationDrug-induced mast cell eradication: A novel approach to treat mast cell activation disorders?
Valent, P., Akin, C., Hartmann, K., Reiter, A., Gotlib, J. R., Sotlar, K., Sperr, W. R., Degenfeld‐Schonburg, L., Smiljkovic, D., Triggiani, M., et al. (2022) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2022.04.003 · Cited by 26 · see citing works
Self-citationMechanisms and management of gastrointestinal symptoms in postural orthostatic tachycardia syndrome
Tu, Y., Abell, T. L., Raj, S. R., & Mar, P. L. (2020) · Neurogastroenterology & Motility · doi:10.1111/nmo.14031 · Cited by 26 · see citing works
A French National Survey on Clotting Disorders in Mastocytosis
Carvalhosa, A., Aouba, A., Damaj, G. L., Canioni, D., Brouzes, C., Gyan, E., Durupt, S., Durieu, I., Cathébras, P. J., Costedoat-Chalumeau, N., et al. (2015) · Medicine · doi:10.1097/md.0000000000001414 · Cited by 24 · see citing works
“INTRODUCTION M astocytosis is a rare clonal hematological disorder characterized by (1) the proliferation and accumulation of neoplastic mast cells in 1 organs (the skin and the bone marrow being predominantly involved) and (2) their unregulated activation/degranulation (ie mast cell mediators release), resulting in m…”
BackgroundKnockdown of the Antiapoptotic Bcl-2 Family Member A1/Bfl-1 Protects Mice from Anaphylaxis
Ottina, E., Lyberg, K., Sochalska, M., Villunger, A., & Nilsson, G. P. (2014) · The Journal of Immunology · doi:10.4049/jimmunol.1400637 · Cited by 20 · see citing works
“In these diseases, mast cell numbers are usually increased, and enhanced levels of mast cell mediators can be measured in plasma or other body fluids (5).”
BackgroundThe role of mast cells in pediatric gastrointestinal disease
Ravanbakhsh, N. (2019) · Annals of Gastroenterology · doi:10.20524/aog.2019.0378 · Cited by 20 · see citing works
“Primary MCAS includes mastocytosis and monoclonal MC activation syndrome; diagnosis is based on objective pathologic findings, as stated in the World Health Organization (WHO) diagnostic criteria [23,69].”
BackgroundInfluentialArock, M., Sotlar, K., Gotlib, J. R., Sperr, W. R., Hartmann, K., Schwartz, L. B., Akin, C., Horny, H., & Valent, P. (2019) · Leukemia & lymphoma/Leukemia and lymphoma · doi:10.1080/10428194.2019.1703974 · Cited by 18 · see citing works
“When MC involvement is documented and the reaction is severe, an MCA syndrome (MCAS) may be diagnosed [18].”
InfluentialSelf-citationNicoloro SantaBarbara, J., & Lobel, M. (2022) · Journal of Health Psychology · doi:10.1177/13591053211014583 · Cited by 17 · see citing works
“blood pressure instability, acid reflux, muscle and bone pain, syncope, brain fog, and flushing (Theoharides et al., 2015; Valent, 2013; Valent et al., 2012).”
BackgroundClinical Approach to Mast Cell Activation Syndrome: A Practical Overview
Matito, A., Escribese, M. M., Longo, N., Mayorga, C., Luengo, O., Pérez‐Gordo, M., Matheu, V. M., Labrador‐Horrillo, M., & Seoane-Reula, E. (2021) · Journal of Investigational Allergology and Clinical Immunology · doi:10.18176/jiaci.0675 · Cited by 16 · see citing works
“…for diagnostic purposes some of the nonspecific symptoms previously included (eg, nasal congestion, headache, neurologic symptoms, and fatigue) [2,11,12] or other manifestations and conditions that lack precision for the diagnosis of MCAS or are not clearly related to MCA (eg, fibromyalgia-like…”
BackgroundSurvey of Mast Cell Mediator Levels from Patients Presenting with Symptoms of Mast Cell Activation
Butterfield, J. H. (2019) · International Archives of Allergy and Immunology · doi:10.1159/000503964 · Cited by 16 · see citing works
Hymenoptera Anaphylaxis and C-kit Mutations: An Unexpected Association
Bonadonna, P., Bonifacio, M., Lombardo, C., & Zanotti, R. (2015) · Current Allergy and Asthma Reports · doi:10.1007/s11882-015-0550-0 · Cited by 14 · see citing works
Valent, P., Akin, C., & Arock, M. (2024) · Current Allergy and Asthma Reports · doi:10.1007/s11882-024-01124-2 · Cited by 13 · see citing works
Self-citationMast Cell Activation Syndrome Update—A Dermatological Perspective
Mihele, D., Nistor, P., Bruma, G., Mitran, C. I., Mitran, M. I., Condrat, C. E., Țovaru, M., Tampa, M. Ş., & Georgescu, S. R. (2023) · Journal of Personalized Medicine · doi:10.3390/jpm13071116 · Cited by 12 · see citing works
Prieto, A., Castells, M., Hansbro, P. M., & Stevens, R. L. (2014) · Immunology and Allergy Clinics of North America · doi:10.1016/j.iac.2014.01.001 · Cited by 12 · see citing works
Pastorello, E. A., Stafylaraki, C., Mirone, C., Preziosi, D., Aversano, M. G., Mascheri, A., Losappio, L. M., Ortolani, V. G. R., Nichelatti, M., & Farioli, L. (2015) · International Archives of Allergy and Immunology · doi:10.1159/000380950 · Cited by 12 · see citing works
Bone marrow tryptase as a possible diagnostic criterion for adult systemic mastocytosis
Livideanu, C. B., Apoil, P. A., Lepage, B., Eischen, M., Laurent, C., Laharrague, P. F., Lamant, L., Tournier, É., Tavitian, S., Pouplard, C., et al. (2015) · Clinical & Experimental Allergy · doi:10.1111/cea.12627 · Cited by 12 · see citing works
Ferrara, A. L., Piscitelli, F., Petraroli, A., Parente, R., Galdiero, M. R., Varricchi, G., Marone, G., Triggiani, M., Marzo, V. D., & Loffredo, S. (2019) · Journal of Immunology Research · doi:10.1155/2019/5836476 · Cited by 12 · see citing works
“Detection of tryptase provides information about MC distribution, numbers, proliferation, and activation status [76] and is, therefore, a major marker of mast cell disorders, including mastocytosis [44, 56, 77, 78].”
BackgroundIn vivo model for mastocytosis: A comparative review
Ranieri, G., Marech, I., Pantaleo, M. A., Piccinno, M., Roncetti, M., Mutinati, M., Rizzo, A., Gadaleta, C. D., Introna, M., Patruno, R., et al. (2014) · Critical Reviews in Oncology/Hematology · doi:10.1016/j.critrevonc.2014.10.010 · Cited by 12 · see citing works
“[100] Valent P.”
BackgroundMicroarray-Based Detection of Allergen-Reactive IgE in Patients with Mastocytosis
Smiljkovic, D., Kiss, R., Lupinek, C., Hoermann, G., Greiner, G., Witzeneder, N., Krajnik, G., Trautinger, F., Vrtala, S., Mittermann, I., et al. (2020) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2020.04.030 · Cited by 12 · see citing works
Self-citationMacy, E. M. (2015) · Current Allergy and Asthma Reports · doi:10.1007/s11882-015-0584-3 · Cited by 10 · see citing works
Fernandes, I. C., Sampaio, R., Moreno, F., Garcia, J. P., Teixeira, M. D. A., Freitas, I. C., Neves, E., Jara‐Acevedo, M., Escribano, L., & Lima, M. (2017) · Allergy Asthma and Clinical Immunology · doi:10.1186/s13223-017-0193-x · Cited by 10 · see citing works
“The European Competence Network on Mastocytosis (ECNM) classified MCAS into three major groups: primary (corresponding to cMCAD), secondary (allergy related), and idiopathic (no clonal MCs and MCA triggers identified) [14].”
BackgroundKhokhar, D. S., & Akin, C. (2019) · Medical Clinics of North America · doi:10.1016/j.mcna.2019.09.002 · Cited by 10 · see citing works
The Italian Mastocytosis Registry: 6-year experience from a Hospital-Based Registry
Merante, S., Ferretti, V. V., Elena, C., Brazzelli, V., Zanotti, R., Neri, I., Magliacane, D., Fortina, A. B., Forer, I., Pastorello, E. A., et al. (2018) · Future Oncology · doi:10.2217/fon-2018-0291 · Cited by 9 · see citing works
Self-citationAllen, S. J., Chazot, P. L., & Dixon, C. J. (2018) · British Journal of Clinical Pharmacology · doi:10.1111/bcp.13578 · Cited by 8 · see citing works
PKC potentiates tyrosine kinase inhibitors STI571 and dasatinib cytotoxic effect.
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Mast cell promotes obesity by activating microglia in hypothalamus
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BackgroundDiagnosis and Treatment of Anaphylaxis in Patients with Mastocytosis
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Self-citationThe roles of mast cells in allergic inflammation and mast cell-related disorders
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“A mast cell activation disorder requires clinical symptomatology that is in keeping with the disorder, a transient, measurable increase in either serum tryptase or other markers of mast cell mediators and a response to agents that interfere with mast cell mediators [7, 8].”
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Oxidative Stress Reduction (Prong-3)
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Bone Marrow Expression of Mast Cell Disorders
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- Authors
- Valent, P.
- Affiliations
- Medical University of Vienna (AT)
- Corresponding author
- Valent, P.
- ORCID
- Valent, P.
- Venue
- Allergy
- Publisher
- Wiley
- Published
- 2013-02-15
- Volume
- 68
- Issue
- 4
- Pages
- 417-424
- ISSN
- 0105-4538 (ISSN-L)
- 1398-9995
- OpenAlex
- W2018681876
- Cited by
- 120
- References
- 69
- Citations by year
- 2026: 4 · 2025: 3 · 2024: 3 · 2023: 5 · 2022: 8 · 2021: 9 · 2020: 7 · 2019: 20 · 2018: 8 · 2017: 8 · 2016: 6 · 2015: 17 · 2014: 17 · 2013: 5
- FWCI
- 1.31
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Allergy (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Universität Wien
- Medizinische Universität Wien
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Immunology and Allergy › Food Allergy and Anaphylaxis Research
- Health Sciences › Medicine › Physiology › Asthma and respiratory diseases
- Keywords
- mast cell activation syndrome, mast cell activation, mastocytosis, KIT mutations, IgE-dependent allergy, anaphylaxis, serum tryptase, mast cell stabilizers, classification
- MeSH terms
- Diagnosis, Differential, Humans, Mast Cells, Mastocytosis, Syndrome, Comorbidity
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