MCAS bibliography | Mast Cell Action

MCAS bibliography and research papers

The following section provides the must-read MCAS bibliography, the latest MCAS research and a more detailed collation of the published literature on MCAS.

The latest bibliography of 'must read' MCAS papers was updated in 2022 and is available here.

The latest MCAS research:

Clustering of clinical symptoms using large language models reveals low diagnostic specificity of proposed alternatives to consensus mast cell activation syndrome criteria (September 2024)
Beyond Confirmed Mast Cell Activation Syndrome: Approaching Patients With Dysautomia and Related Conditions (July 2024)
Mast cell activation syndrome: Current understanding and research needs (June 2024)
Low Prevalence of Idiopathic Mast Cell Activation Syndrome Among 703 Patients With Suspected Mast Cell Disorders (March 2024)
Mast Cells in Autism Spectrum Disorder - The Enigma to Be Solved? (February 2024)
Mast Cell-Targeting Therapies in Mast Cell Activation Syndromes (January 2024)
Predictors of Clonality and Underlying Mastocytosis in Mast Cell Activation Syndromes (January 2024)

Diagnosis

Mast Cell Activation Syndrome: A Research Update

Kathleen Doheny (2023). This is a short informative article explaining the difference between clonal and non-clonal mast cell activation syndrome. It focuses on presentation of each and helpful investigations for a diagnosis. There are also useful links to scientific articles that go more in depth in the detail of the non-clonal form.

A Puzzling Mast Cell Trilogy: Anaphalaxis, MCAS, and Mastocytosis

Theo Gülen (2023). This article outlines the difficulties associated with diagnosing three interrelated conditions; anaphylaxis, MCAS, and mastocytosis. It outlines each in turn, to then discuss their overlap and clinical features of their presentation alongside other mast-cell related disorders. It concludes by calling for future research aimed at identifying new biomarkers to distinguish patients with each disorder and the treatment of patients in specialized centres due to the complexity of the conditions.

Increased Excretion of Mast Cell Mediator Metabolites During Mast Cell Activation Syndrome

Joseph H Butterfield (2023). This is a retrospective cohort study exploring biomarkers in MCAS diagnosis. While an increase in serum tryptase by 20% plus 2 ng/mL is required for an MCAS diagnosis, there is no agreement on what counts as significant rise in urinary metabolites such as prostaglandin D2, histamine, or leukotriene (which are also linked to MCAS episodes). The study finds that the measurements of all three urinary metabolites together is helpful for diagnosis.

Cluster-Analytic Identification of Clinically Meaningful Subtypes in MCAS: The Relevance of Heat and Cold

Tinus Häder, Gerhard J Molderings, Frank Klawonn, Rupert Conrad, Martin Mücke, Julia Sellin (2023). This study is the first to perform a cluster analysis of MCAS patients, classifying them into subgroups based on symptoms and triggers, which could help to personalize treatment and improve patient care. The study concludes that there is utility of a cluster analytic approach and the potential of association analysis to improve the understanding of MCAS subtypes and to personalize the therapy.

Learned cautions regarding antibody testing in mast cell activation syndrome

Lawrence B. Afrin, Tania T. Dempsey and Gerhard J. Molderings (2023). This article discusses the role and limitations of antibody testing in MCAS patients. As MCAS causes abnormal antibody production, it can mimic antibodies that can lead to diagnostic confusion and misdiagnosis of autoimmune or infectious diseases. The article suggests that healthcare professionals should exercise caution with positive antibody test results in MCAS patients. It states that the monitoring of clinical symptoms and repeated testing over time should be used to avoid misdiagnosis caused by misleading results.

KITD816V mutation in blood for the diagnostic screening of systemic mastocytosis and mast cell activation syndromes

Paula Navarro-Navarro, Iván Álvarez-Twose, Alba Pérez-Pons, Ana Henriques, Andrea Mayado, Andrés C. García-Montero, Laura Sánchez-Muñoz, Oscar González-López, Almudena Matito, Carolina Caldas, María Jara-Acevedo, Alberto Orfao (2022). This article discusses the potential utility of investigating KITD816V in genomic DNA to increase diagnostic sensitivity for MCAS. It states that, while KITD816V is a key marker for systemic mastocytosis, it is less detectable in blood for patients with low levels of the mutation, such as patients with c-MCAS. It suggests that tracking the KITD816V mutation over time could help identify patients at higher risk of disease progression.

Mast cell activation syndrome - anesthetic challenges in two different clinical scenarios

Brianna Lide, Shane McGuire, Hong Liu, Cristina Chandler (2022). This is a case report outlining two clinical scenarios of MCAS, aiming to identify possible factors triggering mast cell mediator release. It discusses diagnosis criteria, symptoms, triggers, treatment, premedication and anaphylaxis. It concludes that recognizing and managing MCAS with trigger avoidance and tailored treatment plans is essential for reducing morbidity and achieving optimal outcomes.

Idiopathic mast cell activation syndrome is more often suspected than diagnosed - A prospective real-life study

Thomas Buttgereit, Sophie Gu, Leonor Carneiro-Leão, Annika Gutsche, Marcus Maurer, Frank Siebenhaar (2022). This article details a prospective study of 100 patients with suspected idiopathic MCAS. It investigates the prevalence of diagnostic criteria including increased tryptase and response to MC-targeted treatment. It finds that only 2 of 79 patients had increased tryptase following an episode, and that depression and anxiety disorders were frequent comorbidities. It concludes that, as MCAS was confirmed in only 2% of patients, it is not MC activation that drives symptoms in most patients with suspected MCAS. It calls for further research on the pathogenesis of the disease.

Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal

Peter Valent, Cem Akin, Karin Hartmann, Ivan Alvarez-Twose, Knut Brockow, Olivier Hermine, Marek Niedoszytko, Juliana Schwaab, Jonathan J Lyons, Melody C Carter, Hanneke Oude Elberink, Joseph H Butterfield, Tracy I George, Georg Greiner, Celalettin Ustun, Patrizia Bonadonna, Karl Sotlar, Gunnar Nilsson, Mohamad Jawhar, Frank Siebenhaar, Sigurd Broesby-Olsen, Selim Yavuz, Roberta Zanotti, Magdalena Lange, Boguslaw Nedoszytko, Gregor Hoermann, Mariana Castells, Deepti H Radia, Javier I Muñoz-Gonzalez, Wolfgang R Sperr, Massimo Triggiani, Hanneke C Kluin-Nelemans, Stephen J Galli, Lawrence B Schwartz, Andreas Reiter, Alberto Orfao, Jason Gotlib, Michel Arock, Hans-Peter Horny, Dean D Metcalfe (2021). This article discusses Mastocytosis, and its classification into cutaneous, systemic, and MC sarcoma forms. It highlights the role of the KIT D816V mutation and other genetic markers in determining disease severity and prognosis. The article also proposes updated diagnostic criteria and a global classification system of MC disorders generally, including MCAS.

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

Theo Gülen, Cem Akin, Patrizia Bonadonna, Frank Siebenhaar, Sigurd Broesby-Olsen, Knut Brockow, Marek Niedoszytko, Boguslaw Nedoszytko, Hanneke N G Oude Elberink, Joseph H Butterfield, Wolfgang R Sperr, Ivan Alvarez-Twose, Hans-Peter Horny, Karl Sotlar, Juliana Schwaab, Mohamad Jawhar, Roberta Zanotti, Gunnar Nilsson, Jonathan J Lyons, Melody C Carter, Tracy I George, Olivier Hermine, Jason Gotlib, Alberto Orfao, Massimo Triggiani, Andreas Reiter, Karin Hartmann, Mariana Castells, Michel Arock, Lawrence B Schwartz, Dean D Metcalfe, Peter Valent (2021).

Mast Cell Activation Disorders

Giannetti A, Filice E, Caffarelli C, Ricci G, Pession A. Mast Cell Activation Disorders. Medicina (Kaunas). 2021;57(2):124. Published 2021 Jan 30. doi:10.3390/medicina57020124

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

Gülen T, Akin C, Bonadonna P, et al. Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review [published online ahead of print, 2021 Jun 22]. J Allergy Clin Immunol Pract. 2021;S2213-2198(21)00676-0. doi:10.1016/j.jaip.2021.06.011

A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders

Romantowski J, Górska A, Niedoszytko M, et al. A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders. Int J Mol Sci. 2021;22(3):1454. Published 2021 Feb 1. doi:10.3390/ijms22031454

Clinical Approach to Mast Cell Activation Syndromes: A Practical Overview

Matito A, Escribese MM, Longo N, et al. Clinical Approach to Mast Cell Activation Syndromes: A Practical Overview [published online ahead of print, 2021 Feb 4]. J Investig Allergol Clin Immunol. 2021;0. doi:10.18176/jiaci.0675

New developments in the field of mastocytosis and mast cell activation syndromes: a summary of the Annual Meeting of the European Competence Network on Mastocytosis (ECNM) 2019

Arock M, Sotlar K, Gotlib J, et al. New developments in the field of mastocytosis and mast cell activation syndromes: a summary of the Annual Meeting of the European Competence Network on Mastocytosis (ECNM) 2019. Leuk Lymphoma. 2020;61(5):1075-1083. doi:10.1080/10428194.2019.1703974

Mast Cell Activation: When the Whole Is Greater than the Sum of Its Parts

Khokhar D, Akin C. Mast Cell Activation: When the Whole Is Greater than the Sum of Its Parts. Med Clin North Am. 2020;104(1):177-187. doi:10.1016/j.mcna.2019.09.002

Mast cell activation syndromes - evaluation of current diagnostic criteria and laboratory tools in clinical practice (Review)

Leru PM, Anton VF, Ureche C, Zurac S, Bratu O, Neagoe CD. Mast cell activation syndromes - evaluation of current diagnostic criteria and laboratory tools in clinical practice (Review). Exp Ther Med. 2020;20(3):2348-2351. doi:10.3892/etm.2020.8947

Diagnosis, Classification and Management of Mast Cell Activation Syndromes (MCAS) in the Era of Personalized Medicine

Valent P, Akin C, Nedoszytko B, et al. Diagnosis, Classification and Management of Mast Cell Activation Syndromes (MCAS) in the Era of Personalized Medicine. Int J Mol Sci. 2020;21(23):9030. Published 2020 Nov 27. doi:10.3390/ijms21239030

How to diagnose mast cell activation syndrome: practical considerations

Romantowski J, Górska A, Lange M, Nedoszytko B, Gruchała-Niedoszytko M, Niedoszytko M. How to diagnose mast cell activation syndrome: practical considerations. Pol Arch Intern Med. 2020;130(4):317-323. doi:10.20452/pamw.15212

Expanding spectrum of mast cell activation disorders: monoclonal and idiopathic mast cell activation syndromes

Picard M, Giavina-Bianchi P, Mezzano V, Castells M. Expanding spectrum of mast cell activation disorders: monoclonal and idiopathic mast cell activation syndromes. Clin Ther. 2013;35(5):548-562. doi:10.1016/j.clinthera.2013.04.001

Non-Clonal Mast Cell Activation: A Growing Body of Evidence

Hamilton MJ. Nonclonal Mast Cell Activation Syndrome: A Growing Body of Evidence. Immunol Allergy Clin North Am. 2018;38(3):469-481. doi:10.1016/j.iac.2018.04.002

Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome

Valent P, Akin C, Bonadonna P, et al. Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome. J Allergy Clin Immunol Pract. 2019;7(4):1125-1133.e1. doi:10.1016/j.jaip.2019.01.006

Recent advances in our understanding of mast cell activation – or should it be mast cell mediator disorders?

Theoharides TC, Tsilioni I, Ren H. Recent advances in our understanding of mast cell activation - or should it be mast cell mediator disorders?. Expert Rev Clin Immunol. 2019;15(6):639-656. doi:10.1080/1744666X.2019.1596800

The Many Faces of Mast Cell Disorders—A House of Mirrors?

Golden DBK. The Many Faces of Mast Cell Disorders-A House of Mirrors?. J Allergy Clin Immunol Pract. 2019;7(4):1139-1141. doi:10.1016/j.jaip.2019.02.003

Mast cell activation syndrome: Importance of consensus criteria and call for research

Valent P, Akin C, Bonadonna P, et al. Mast cell activation syndrome: Importance of consensus criteria and call for research. J Allergy Clin Immunol. 2018;142(3):1008-1010. doi:10.1016/j.jaci.2018.06.004.

Spectrum of mast cell activation disorders

Petra AI, Panagiotidou S, Stewart JM, Conti P, Theoharides TC. Spectrum of mast cell activation disorders. Expert Rev Clin Immunol. 2014;10(6):729-739. doi:10.1586/1744666X.2014.906302

Mast Cell Activation Disorders

Akin C. Mast cell activation disorders. J Allergy Clin Immunol Pract. 2014;2(3):252-258. doi:10.1016/j.jaip.2014.03.007

Is It Really Fibromyalgia? Recognizing Mast Cell Activation, Orthostatic Tachycardia, and Hypermobility

Russek, Leslie. (2018). Is It Really Fibromyalgia? Recognizing Mast Cell Activation, Orthostatic Tachycardia, and Hypermobility.

Doctor, I Think I Am Suffering from MCAS: Differential Diagnosis and Separating Facts from Fiction

Valent P, Akin C. Doctor, I Think I Am Suffering from MCAS: Differential Diagnosis and Separating Facts from Fiction. J Allergy Clin Immunol Pract. 2019;7(4):1109-1114. doi:10.1016/j.jaip.2018.11.045

AAAAI Mast Cell Disorders Committee Work Group Report: Mast Cell Activation Syndrome (MCAS) Diagnosis and Management

Weiler CR, Austen KF, Akin C, et al. AAAAI Mast Cell Disorders Committee Work Group Report: Mast cell activation syndrome (MCAS) diagnosis and management. J Allergy Clin Immunol. 2019;144(4):883-896. doi:10.1016/j.jaci.2019.08.023

A concise, practical guide to diagnostic assessment for mast cell activation disease

Afrin LB, Molderings GJ. A concise, practical guide to diagnostic assessment for mast cell activation disease. World J Hematol 2014; 3(1): 1-17 [DOI: 10.5315/wjh.v3.i1.1]

Diagnosis of mast cell activation syndrome: a global "consensus-2".

Afrin LB, Ackerley MB, Bluestein LS, et al. Diagnosis of mast cell activation syndrome: a global "consensus-2". Diagnosis (Berl). 2020;8(2):137-152. Published 2020 Apr 22. doi:10.1515/dx-2020-0005

Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options

Molderings GJ, Brettner S, Homman J et al. Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options

Serum mast cell tryptase measurements: Sensitivity and specificity for a diagnosis of anaphylaxis in emergency department patients with shock or hypoxaemia

Francis A, Fatovich DM, Aren. Serum mast cell tryptase measurements: Sensitivity and specificity for a diagnosis of anaphylaxis in emergency department patients with shock or hypoxaemia. Emerg Med Australas. 2017 Nov 2.

Mast cell activation syndromes

Akin C. Mast cell activation syndromes. J Allergy Clin Immunol. 2017 Aug;140(2):349-355. PMID: 28780942 DOI: 10.1016/j.jaci.2017.06.007

Characterization of Mast Cell Activation Syndrome

Mast cell activation syndrome (MCAS), a recently recognized nonneoplastic mast cell disease driving chronic multisystem inflammation and allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population.

Utilities of Various Mast Cell Mediators in Diagnosing Mast Cell Activation Syndrome

Nicolas Zenker and Lawrence B Afrin,Utilities of Various Mast Cell Mediators in Diagnosing Mast Cell Activation Syndrome Blood 2015 126:5174

Mast Cells, Mastocytosis, and Related Disorders

Theoharides TC, Valent P, Akin C. Mast Cells, Mastocytosis, and Related Disorders. N Engl J Med. 2015;373(2):163-72.

Characterization Of Common Blood Test Abnormalities Potentially Aiding Diagnosis Of Mast Cell Activation Syndrome: A Preliminary Analysis

Lawrence B Afrin, Zhuang Wan and Elizabeth G Hill Characterization Of Common Blood Test Abnormalities Potentially Aiding Diagnosis Of Mast Cell Activation Syndrome: A Preliminary Analysis Blood 2013 122:5240;

Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal.

Valent P, Akin C, Arock M 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(3):215-25.

Clinical Manifestations

Cluster-Analytic Identification of Clinically Meaningful Subtypes in MCAS: The Relevance of Heat and Cold

Learned cautions regarding antibody testing in mast cell activation syndrome

Health-related quality of life and health literacy in patients with systemic mastocytosis and mast cell activation syndrome

Tobias Jürgen Schmidt, Julia Sellin, Gerhard J Molderings, Rupert Conrad, Martin Mücke (2022). This article assesses the health-related quality of life (HRQOL) and health literacy of people suffering from Systemic Mastocytosis (SM) and Mast Cell Activation Syndrome (MCAS). It finds that SM and MCAS have a significant impact on the quality of life of individuals, and patients with SM typically receive significantly more information from physicians about their disease. Their results demonstrate the importance of patients’ level of information, as even slight improvements in health literacy can have a positive effect on their quality of life.

Safety of COVID-19 vaccination in patients with mastocytosis and monoclonal mast cell activation syndrome

Rayan Kaakati, Dilawar Khokhar, Cem Akin (2021).

mRNA COVID-19 vaccine is well tolerated in patients with cutaneous and systemic mastocytosis with mast cell activation symptoms and anaphylaxis

Tiago Azenha Rama, André Moreira, Mariana Castells (2021).

Persistent Antiphospholipid Antibodies, Mast Cell Activation Syndrome, Postural Orthostatic Tachycardia Syndrome and Post-COVID Syndrome: 1 Year On

Jill R Schofield (2021)

Mast Cell Activation Syndrome: A Primer for the Gastroenterologist

Mast Cell Activation Syndrome: A Primer for the Gastroenterologist Leonard B. Weinstock, Laura A. Pace, Ali Rezaie, Lawrence B. Afrin & Gerhard J. Molderings

Mast cell activation syndrome: Myths and realities

Zhang S, Bernstein JA. Mast cell activation syndrome: Myths and realities. Allergy Asthma Proc. 2021;42(3):198-204. doi:10.2500/aap.2021.42.210012

A case of neuropathic pain in monoclonal mast cell activation syndrome

Bay JL, Sedarsky KE, Petersen MM. A case of neuropathic pain in monoclonal mast cell activation syndrome. Ann Allergy Asthma Immunol. 2018;120(5):543-544. doi:10.1016/j.anai.2018.02.019

Gastrointestinal Involvement in Mast Cell Activation Disorder

Hsieh FH. Gastrointestinal Involvement in Mast Cell Activation Disorders. Immunol Allergy Clin North Am. 2018;38(3):429-441. doi:10.1016/j.iac.2018.04.008

A Review of the Dermatologic Symptoms of Idiopathic Mast Cell Activation Syndrome

Qureshi AA, Friedman AJ. A Review of the Dermatologic Symptoms of Idiopathic Mast Cell Activation Syndrome. J Drugs Dermatol. 2019;18(2):162-168.

Mast Cell Activation Syndrome: is Anaphylaxis part of the phenotype?

Sabato V, Michel M, Blank U, Ebo DG, Vitte J. Mast cell activation syndrome: is anaphylaxis part of the phenotype? A systematic review [published online ahead of print, 2021 Jul 20]. Curr Opin Allergy Clin Immunol. 2021;10.1097/ACI.0000000000000768. doi:10.1097/ACI.0000000000000768

Fatal anaphylaxis following a hornet sting in a yellow jacket venom-sensitized patient with undetected monoclonal mast cell activation syndrome and without previous history of a systemic sting reaction

Blank S, Pehlivanli S, Methe H, et al. Fatal anaphylaxis following a hornet sting in a yellow jacket venom-sensitized patient with undetected monoclonal mast cell activation syndrome and without previous history of a systemic sting reaction. J Allergy Clin Immunol Pract. 2020;8(1):401-403.e2. doi:10.1016/j.jaip.2019.06.021

Idiopathic Anaphylaxis: A Form of Mast Cell Activation Syndrome

Giannetti MP, Akin C, Castells M. Idiopathic Anaphylaxis: A Form of Mast Cell Activation Syndrome. J Allergy Clin Immunol Pract. 2020;8(4):1196-1201. doi:10.1016/j.jaip.2019.10.048

Tinnitus in MCAS

Weinstock LB, Brook JB, Blasingame KE, Kaleem Z, Afrin LB, et al. (2021) Tinnitus in mast cell activation syndrome: A prospective survey of 114 patients. J Otolaryngol Neurotol Res, 4(1): 92-96.

White matter abnormalities are also repeatedly present in patients with systemic mast cell activation syndrome

Haenisch B, Molderings GJ. White matter abnormalities are also repeatedly present in patients with systemic mast cell activation syndrome. Transl Psychiatry. 2018;8(1):95. Published 2018 May 10. doi:10.1038/s41398-018-0143-5

Mast Cell Activation Syndrome Masquerading as Agranulocytosis

Afrin LB. Mast cell activation syndrome masquerading as agranulocytosis. Mil Med. 2012;177(1):113-117. doi:10.7205/milmed-d-11-00111

Idiopathic Mast Cell activation syndrome With associated salicylate Intolerance

Rechenauer T, Raithel M, Götze T, et al. Idiopathic Mast Cell Activation Syndrome With Associated Salicylate Intolerance. Front Pediatr. 2018;6:73. Published 2018 Mar 27. doi:10.3389/fped.2018.00073

Mast cell activation disease: An underappreciated cause of neurologic and psychiatric symptoms and diseases

Afrin LB, Pöhlau D, Raithel M, et al. Mast cell activation disease: An underappreciated cause of neurologic and psychiatric symptoms and diseases. Brain Behav Immun. 2015;50:314-321. doi:10.1016/j.bbi.2015.07.002

Mast cell activation syndrome in pregnancy, delivery, postpartum and lactation: a narrative review

Dorff SR, Afrin LB. Mast cell activation syndrome in pregnancy, delivery, postpartum and lactation: a narrative review. J Obstet Gynaecol. 2020;40(7):889-901. doi:10.1080/01443615.2019.1674259

Syncope as a manifestation of mast cell activation disorder

Perales Chordá C, Fabregat Nebot S, Moral Moral P, Jarque Ramos I, Hernández Fernandez de Rojas D. Syncope as a manifestation of mast cell activation disorder. Ann Allergy Asthma Immunol. 2015;114(2):153-154. doi:10.1016/j.anai.2014.11.019

Cardiovascular symptoms in patients with systemic mast cell activation disease

Kolck UW, Haenisch B, Molderings GJ. Cardiovascular symptoms in patients with systemic mast cell activation disease. Transl Res. 2016;174:23-32.e1. doi:10.1016/j.trsl.2015.12.012

Mast cell activation disease and the modern epidemic of chronic inflammatory disease

Afrin LB. Mast cell activation disease and the modern epidemic of chronic inflammatory disease. Transl Res. 2016;174:33-59. doi:10.1016/j.trsl.2016.01.003

Mast cell activation syndrome: High frequency of skin manifestations and anaphylactic shock

Casassa EA, Mailhol C, Tournier E, et al. Mast cell activation syndrome: High frequency of skin manifestations and anaphylactic shock. Allergol Int. 2019;68(1):119-121. doi:10.1016/j.alit.2018.07.003

Symptoms of mast cell activation syndrome in functional gastrointestinal disorders

Wilder-Smith CH, Drewes AM, Materna A, Olesen SS. Symptoms of mast cell activation syndrome in functional gastrointestinal disorders. Scand J Gastroenterol. 2019;54(11):1322-1325. doi:10.1080/00365521.2019.1686059

The Mastocytosis Society Survey on Mast Cell Disorders: Part 2-Patient Clinical Experiences and Beyond

Russell N, Jennings S, Jennings B, et al. The Mastocytosis Society Survey on Mast Cell Disorders: Part 2-Patient Clinical Experiences and Beyond. J Allergy Clin Immunol Pract. 2019;7(4):1157-1165.e6. doi:10.1016/j.jaip.2018.07.032

Mast Cell Activation Syndrome Mimicking Breast Cancer: Case Report With Pathophysiologic Considerations

Molderings GJ, Knüchel-Clarke R, Hertfelder HJ, Kuhl C. Mast Cell Activation Syndrome Mimicking Breast Cancer: Case Report With Pathophysiologic Considerations. Clin Breast Cancer. 2018;18(3):e271-e276. doi:10.1016/j.clbc.2017.12.004

Symptoms of Mast Cell Activation: The Patient Perspective

Jennings SV, Slee VM, Finnerty CC, Hempstead JB, Bowman AS. Symptoms of Mast Cell Activation: The Patient Perspective [published online ahead of print, 2021 Jul 13]. Ann Allergy Asthma Immunol. 2021;S1081-1206(21)00494-4. doi: 10.1016/j.anai.2021.07.004

Nonhistaminergic idiopathic angioedema may be a presentation of mast cell activation syndrome.

Afrin LB. Nonhistaminergic idiopathic angioedema may be a presentation of mast cell activation syndrome. J Investig Allergol Clin Immunol. 2013;23(3):212.

Immunology and clinical manifestations of non-clonal mast cell activation syndrome

Cardet JC, Castells MC, Hamilton MJ. Immunology and clinical manifestations of non-clonal mast cell activation syndrome. Curr Allergy Asthma Rep. 2013;13(1):10-18. doi:10.1007/s11882-012-0326-8

Mast Cell Activation Syndrome: A Primer for the Gastroenterologist

Weinstock LB, Pace LA, Rezaie A, Afrin LB, Molderings GJ. Mast Cell Activation Syndrome: A Primer for the Gastroenterologist. Dig Dis Sci. 2021;66(4):965-982. doi:10.1007/s10620-020-06264-9

Treatment

Fluoxetine retrains allergic inflammation by targeting an FcɛRI-ATP positive feedback loop in mast cells

Tamara T Haque, Marcela T Taruselli, Sydney A Kee, Jordan M Dailey, Neha Pondicherry, Paula A Gajewski-Kurdziel, Matthew P Zellner, Daniel J Stephenson, H Patrick MacKnight, David B Straus, Roma Kankaria, Kaitlyn G Jackson, Alena P Chumanevich, Yoshihiro Fukuoka, Lawrence B Schwartz, Randy D Blakely, Carole A Oskeritzian, Charles E Chalfant, Rebecca K Martin, John J Ryan (2023). This article outlines a study on mice that tests the effects of the SSRI fluoxetine on IgE-induced activation of mast cells. It discusses how fluoxetine suppresses mast cell activation and ATP secretion, which amplifies IgE responses. The article concludes by suggesting that fluoxetine is a promising candidate for repurposing allergy treatment.

Mast Cells as a Target - A Comprehensive Review of Recent Therapeutic Approaches

Joanna Baran, Anna Sobiepanek, Anna Mazurkiewicz-Pisarek, Marta Rogalska, Aleksander Gryciuk, Lukasz Kuryk, Soman N Abraham, Monika Staniszewska (2023). This article provides a comprehensive overview of therapeutic approaches targeting mast cells, making it highly relevant to the treatment theme. It discusses the role of mast cells in the immune system, their overactivity in pathological states, and various therapeutic strategies to manage these conditions.

How can a dietician help support those with Mast Cell Activation Syndrome?

Chloe Hall (2023). This article gives an overview of dietary triggers associated with MCAS and dietary recommendations for the management of MCAS symptoms. It is targeted to dieticians, outlining their role in identifying dietary triggers in MCAS, safely re-introducing foods where anaphylaxis has occurred in the past, and in fostering a healthy relationship between MCAS patients and food.

Mast Cell Diseases in Practice and Research: Issues and Perspectives Raised by Patients and Their Recommendations to the Scientific Community and Beyond

Susan V. Jennings, Celeste C. Finnerty, Jessica S. Hobart, Mercedes Martín-Martínez, Kristin A. Sinclairf, Valerie M. Slee, Julie Agopian, Cem Akin, Ivan Álvarez-Twose, Patrizia Bonadonna, Angela S. Bowman, Knut Brockow, Horia Bumbea, Claudia de Haroo, Jie Shen Fok, Karin Hartmann, Nicole Hegmanns, Olivier Hermine, Monika Kalisiak, Constance H. Katelaris, Jacqueline Kurzs, Patrizia Marcis, David Mayne, David Mendoza, Alain Moussy, Genija Mudretzkyjz, Nicoleta Nidelea Vaiaaa, Marek Niedoszytko, Hanneke Oude Elberink, Alberto Orfao, Deepti H. Radia, Sophie Rosenmeierz ∙ Eugenia Ribadad ∙ Waltraud Schinhofenff ∙ Juliana Schwaab, Frank Siebenhaar, Massimo Triggiani, Giuseppe Tripodox, Rocio Velazquezo, Yvon Wielinkkk, Friedrich Wimazal, Timo Yigitff, Celia Zubrinich, Peter Valent (2022).This article focuses on the perspectives of those with mast cell diseases (namely mastocytosis and MCAS). It details a project aiming to expand our understanding of patient needs and to propose actions that address those needs. Issues reported include struggles relating to the rarity of mast cell diseases, the impact of the diseases on quality of life, the diagnostic process and the need for further research. It also identifies possible solutions to these challenges.

Drug-induced mast cell eradication: A novel approach to treat mast cell activation disorders?

Peter Valent, Cem Akin, Karin Hartmann, Andreas Reiter, Jason Gotlib, Karl Sotlar, Wolfgang R. Sperr, Lina Degenfeld-Schonburg, Dubravka Smiljkovic, Massimo Triggiani, Hans-Peter Horny, Michel Arock, Stephen J. Galli, Dean D. Metcalfe (2022). This article examines the potential of KIT-targeting tyrosine kinase inhibitors (TKIs) in treating MCAS by blocking mast cell activation. It highlights that while TKIs like avapritinib show promise in targeting KIT D816V-positive mast cells, their effectiveness in MCAS remains unclear. The authors suggest more research is needed to determine the long-term safety and efficacy of these treatments for MCAS patients.

High FODMAP diet causes barrier loss via lipopolysaccharide-mediated mast cell activation

Prashant Singh, Gintautas Grabauskas, Shi-Yi Zhou, Jun Gao, Yawen Zhang, Chung Owyang (2021). This article discusses how a high FODMAP (HFM) diet induces mast cell activation and colonic barrier dysfunction in rodent models and patients with IBS-D. It highlights the critical role of TLR4-dependent mast cell activation in FODMAP-induced barrier loss and the potential benefits of a low FODMAP (LFM) diet in reducing mast cell activation and improving colonic barrier function. The study provides new insights into the microbiome’s contribution to mast cell activation and its relevance for managing gastroinstestinal symptoms in IBS-D.

Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association

Ritsuko Kohno, David S Cannom, Brian Olshansky, Shijun Cindy Xi, Darshan Krishnappa, Wayne O Adkisson, Faye L Norby, Artur Fedorowski, David G Benditt (2021). This study examines the frequency with which findings suggesting mast cell activation (MCA) disorder occurred in patients diagnosed with postural orthostatic tachycardia syndrome (POTS). It notes a broader symptom profile in MCA cases, including allergic and gastrointestinal issues. It identifies prostaglandins combined with other markers, such as histamine, as useful but not definitive diagnostic indicators and concludes that MCA should be considered in POTS-like cases with atypical symptoms.

Safe administration of chemotherapy in mast cell activation syndrome

M P Lythgoe, J Krell, I A McNeish, L Tookman (2021). This article details a case report of a 59-year-old MCAS patient with endometrial cancer who received chemotherapy, with the aim to provide a framework for safer chemotherapy administration. It discusses the challenges of administering chemotherapy in patients with MCAS, highlighting hypersensitivity risks of carboplatin and paclitaxel, the role of desensitization protocols, and the need for tailored strategies to safely treat high-risk patients.

Mast Cell Activation Syndrome: A Primer for the Gastroenterologist

Leonard B Weinstock, Laura A Pace, Ali Rezaie, Lawrence B Afrin, Gerhard J Molderings (2021). This article reviews gastrointestinal comorbidities in MCAS, including links to conditions such as Postural Orthostatic Tachycardia Syndrome (POTS) and hypermobile Ehlers_Danlos Syndrome (hEDS). It finds that gastrointestinal symptoms in MCAS are inflluenced by mast cell activation, autonomic dysfunction, and connective tissue abnormalities, requiring comprehensive diagnostic and therapeutic approaches.

Effect of Dietary Fiber and Metabolites on Mast Cell Activation and Mast Cell-Associated Diseases

Jelle Folkerts, Ralph Stadhouders, Frank A Redegeld, See-Ying Tam, Rudi W Hendriks, Stephen J Galli, Marcus Maurer (2018). This article provides an overview of current understandings surrounding the effects of dietary fibre on the regulation of mast cell activity and mast cell-associated diseases. It discusses the potential therapeutic effects of fibre and its metabolites (including short-chain fatty acids) on mast-cell associated airway inflammation and allergic diseases. It proposes further research to explore the role of dietary fibre in preventing and treating conditions associated with mast cell activation.

Inhibitory effects of curcumin on passive cutaneous anaphylactoid response and compound 48/80-induced mast cell activation

Yun-Ho Choi 1, Guang-Hai Yan, Ok Hee Chai, Chang Ho Song (2010) This article discusses the inhibitory effects of curcumin (the active component of turmeric) on mast cell activation and histamine release in mast cell-mediated allergic responses. It highlights curcumin’s mechanisms, including suppression of calcium influx and enhancement of intracellular cAMP levels, which reduce mast cell degranulation and histamine release. The findings suggest curcumin’s potential therapeutic role in managing mast cell-associated allergic diseases.

Mast Cell Activation Syndrome and Mastocytosis: Initial Treatment Options and Long-Term Management

Castells M, Butterfield J. Mast Cell Activation Syndrome and Mastocytosis: Initial Treatment Options and Long-Term Management. J Allergy Clin Immunol Pract. 2019;7(4):1097-1106. doi:10.1016/j.jaip.2019.02.002

Omalizumab effectively prevents recurrent refractory anaphylaxis in a patient with monoclonal mast cell activation syndrome.

Jagdis A, Vadas P. Omalizumab effectively prevents recurrent refractory anaphylaxis in a patient with monoclonal mast cell activation syndrome. Ann Allergy Asthma Immunol. 2014;113(1):115-116. doi:10.1016/j.anai.2014.05.001

Timing of response and long term efficacy of Omalizumab in non-clonal mast cell activation syndrome: A case series.

Caminati M, Olivieri E, Nalin F, et al. Timing of response and long term efficacy of Omalizumab in non-clonal Mast Cell Activation Syndrome: A case series. Eur J Intern Med. 2020;71:104-106. doi:10.1016/j.ejim.2019.11.010

Mast Cell disorders and idiopathic anaphylaxis: Evaluation and Management

Khalid MB, Lieberman P. Mast cell disorders and idiopathic anaphylaxis: Evaluation and management. Allergy Asthma Proc. 2020;41(2):90-98. doi:10.2500/aap.2020.41.190023

Successful treatment of mast cell activation syndrome with sunitinib

Afrin LB, Cichocki FM, Patel K, Molderings GJ. Successful treatment of mast cell activation syndrome with sunitinib. Eur J Haematol. 2015;95(6):595-597. doi:10.1111/ejh.12606

Successful targeted treatment of mast cell activation syndrome with tofacitinib

Afrin LB, Fox RW, Zito SL, Choe L, Glover SC. Successful targeted treatment of mast cell activation syndrome with tofacitinib. Eur J Haematol. 2017;99(2):190-193. doi:10.1111/ejh.12893

Generalized Pruritus Relieved by NSAIDs in the Setting of Mast Cell Activation Syndrome

Kesterson K, Nahmias Z, Brestoff JR, Bodet ND, Kau A, Kim BS. Generalized pruritus relieved by NSAIDs in the setting of mast cell activation syndrome. J Allergy Clin Immunol Pract. 2018;6(6):2130-2131. doi:10.1016/j.jaip.2018.03.002

Hydroxychloroquine as a novel therapeutic approach in mast cell activation diseases

Espinosa E, Valitutti S, Laroche M, et al. Hydroxychloroquine as a novel therapeutic approach in mast cell activation diseases. Clin Immunol. 2018;194:75-79. doi:10.1016/j.clim.2018.07.004

Successful treatment of idiopathic mast cell activation syndrome with low-dose Omalizumab

Berry R, Hollingsworth P, Lucas M. Successful treatment of idiopathic mast cell activation syndrome with low-dose Omalizumab. Clin Transl Immunology. 2019;8(10):e01075. Published 2019 Sep 30. doi:10.1002/cti2.1075

Utility of hydroxyurea in mast cell activation syndrome

Afrin LB. Utility of hydroxyurea in mast cell activation syndrome. Exp Hematol Oncol. 2013;2(1):28. Published 2013 Oct 9. doi:10.1186/2162-3619-2-28

Successful treatment of postural orthostatic tachycardia and mast cell activation syndromes using naltrexone, immunoglobulin and antibiotic treatment

Weinstock LB, Brook JB, Myers TL, Goodman B. Successful treatment of postural orthostatic tachycardia and mast cell activation syndromes using naltrexone, immunoglobulin and antibiotic treatment. BMJ Case Rep. 2018;2018:bcr2017221405. Published 2018 Jan 11. doi:10.1136/bcr-2017-221405

Selective Response to Omalizumab in a Patient With Concomitant ncMCASand POTS: What Does it Teach us About the Underlying Disease?

Kacar M, Denman S, Savic S. Selective Response to Omalizumab in a Patient With Concomitant ncMCAS and POTS: What Does it Teach us About the Underlying Disease?. J Investig Allergol Clin Immunol. 2018;28(4):261-263. doi:10.18176/jiaci.0251

Management of a Parturient with Mast Cell Activation Syndrome: An Anesthesiologist’s Experience

Kumaraswami, Sangeeta & Farkas, Gabriel. (2018). Management of a Parturient with Mast Cell Activation Syndrome: An Anesthesiologist’s Experience. Case Reports in Anesthesiology. 2018. 1-5. 10.1155/2018/8920921.

Continuous diphenhydramine infusion and imatinib for KIT-D816V-negative mast cell activation syndrome: A case report

Malik F, Ali N, Jafri SIM, et al. Continuous diphenhydramine infusion and imatinib for KIT-D816V-negative mast cell activation syndrome: a case report. J Med Case Rep. 2017;11(1):119. Published 2017 Apr 24. doi:10.1186/s13256-017-1278-3

Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes

González-de-Olano D, Matito A, Orfao A, Escribano L. Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes. F1000Res. 2016;5:2666. Published 2016 Nov 14. doi:10.12688/f1000research.9565.1

21st Century Mast Cell Stabilisers

Finn DF, Walsh JJ. Twenty-first century mast cell stabilizers. Br J Pharmacol. 2013;170(1):23-37. doi:10.1111/bph.12138

A Practical Guide for Treatment of Pain in Patients with Systemic Mast Cell Activation Disease

Wirz S, Molderings GJ. A Practical Guide for Treatment of Pain in Patients with Systemic Mast Cell Activation Disease. Pain Physician. 2017 Sep;20(6):E849-E861.

Pharmacological treatment options for mast cell activation

Molderings GJ, Haenisch B, Brettner S et al. Pharmacological treatment options for mast cell activation. Naunyn Schmiedebergs Arch Pharmacol. 2016 Jul;389(7):671-94

Utility of Continuous Diphenhydramine Infusion in Severe Mast Cell Activation Syndrome

Lawrence B Afrin; Utility of Continuous Diphenhydramine Infusion in Severe Mast Cell Activation Syndrome; Blood 2015 126:5194

H1-antihistamines for primary mast cell activation syndromes: a systematic review

4 Nurmatov UB, Rhatigan E, Simons FE, Sheikh A. H1-antihistamines for primary mast cell activation syndromes: a systematic review. Allergy. 2015;70(9):1052-61.

The presentation, diagnosis and treatment of mast cell activation syndrome

.2. Afrin LD. 2013 Nova Science Publishers, Mast Cells: Phenotypic Features, Biological Functions and Role in Immunity. Chapters. Presentation, Diagnosis, and Management of Mast Cell Activation Syndrome

Intravenous infusion of ascorbic acid decreases serum histamine concentrations in patients with allergic and non-allergic diseases

Hagel AF, Layritz CM, Hagel WH, et al. Intravenous infusion of ascorbic acid decreases serum histamine concentrations in patients with allergic and non-allergic diseases. Naunyn Schmiedebergs Arch Pharmacol. 2013 Sep;386(9):789-93.

The 1,4-benzodiazepine Ro5-4864 (4-chlorodiazepam) suppresses multiple pro-inflammatory mast cell effector functions.

Yousefi OS, Wilhelm T, Maschke-Neuß K, et al. The 1,4-benzodiazepine Ro5-4864 (4-chlorodiazepam) suppresses multiple pro-inflammatory mast cell effector functions. Cell Commun Signal. 2013 Feb 20;11(1):13.

Treatment of mast cells with carbon dioxide suppresses degranulation via a novel mechanism involving repression of increased intracellular calcium levels

Strider JW, Masterson CG, Durham PL. Treatment of mast cells with carbon dioxide suppresses degranulation via a novel mechanism involving repression of increased intracellular calcium levels. Allergy. 2011 Mar;66(3):341-50.

Comorbidities

Neuropsychiatric Manifestations of Mast Cell Activation Syndrome and Response to Mast-Cell-Directed Treatment: A Case Series

Leonard B. Weinstock, Renee M. Nelson and Svetlana Blitshteyn (2023). This is a small study that explores the comorbidity of psychiatric conditions and MCAS. The study includes 8 patients with both a psychiatric and MCAS diagnosis and highlights how, treating MCAS improved the psychiatric symptoms. The study highlights how psychiatric patient's refractory to standard therapy that also have systemic symptoms should be assessed for MCAS. It calls for more studies to investigate the prevalence of MCAS in psychiatric patients not responding to standard treatments to get a better picture of this association and the potential therapeutic benefits for the patients.

Immunological dysfunction and mast cell activation syndrome in long COVID

Stevent Sumantri, Iris Rengganis (2023). This article explores the similarities between long COVID and MCAS; long COVID-19 is persistent inflammatory state that causes abnormal mast cell activation and the release of inflammatory cytokines. This connection opens therapeutic opportunities for patients with long COVID that have clinical symptoms consistent with MCAS.

Mast Cells and Vitamin D Status: A Clinical and Biological Link in the Onset of Allergy and Bone Diseases

Giuseppe Murdaca, Alessandro Allegra, Alessandro Tonacci, Caterina Musolino, Luisa Ricciardi, Sebastiano Gangemi (2022). This article analyses the relationships between mast cells and vitamin D, highlighting their roles in allergic diseases, bone metabolism, and skin conditions. It discusses how vitamin D influences mast cell activation, degranulation, and pro-inflammatory mediator release. Therefore, the findings suggest that vitamin D is a potential therapeutic strategy for managing mast cell driven conditions such as MCAS.

Depression, psychosocial correlates, and psychosocial resources in individuals with mast cell activation syndrome

Jennifer Nicoloro SantaBarbara, Marci Lobel (2022). This article examines the psychological and emotional experiences of 125 participants with MCAS. Clinical depression was found to be common in the sample (64% prevalence), as well as feelings of loneliness and defectiveness, which contributed to high illness intrusiveness. The study concludes that while stigma and self-efficacy overlap with other variables, loneliness and disease-specific stressors emerge as significant risks, and optimism acts as a resilience factor.

Mast cell activation and the link with long COVID

Soumya Arun, Abbie Storan, Bethan Myers (2022). This article provides a comprehensive overview of MCAS biomarkers, diagnosis and management before relating the disease to long COVID. It discusses the overlap in symptoms, the potential exacerbation or incitement of MCAS by COVID-19 infection, common environmental triggers, and similarities in relapse-remission cycles in people with long COVID and people with MCAS. It also discusses improvements with similar medications in both diseases, but emphasises that despite these similarities, other underlying pathologies may also be associated with long COVID aside from mast cell disorders.

Mast Cell Activation Syndrome in COVID-19 and Female Reproductive Function: Theoretical Background vs. Accumulating Clinical Evidence

Dariusz Szukiewicz, Piotr Wojdasiewicz, Mateusz Watroba, Grzegorz Szewczyk (2022). This article discusses the importance of mast cells in the female reproductive system and discusses the mechanism of potential disorders related to MCAS. While it suggests that COVID-19 hyperinflammation may involve MCAS, it finds that current evidence does not indicate a significant impact on female reproductive function

Mast cell activation disease and immunoglobulin deficiency in patients with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorder

Isabelle Brock, Walter Prendergast, Anne Maitland (2021).

Mast cell activation may explain many cases of chemical intolerance

Claudia S. Miller, Raymond F. Palmer, Tania T. Dempsey, Nicholas A. Ashford & Lawrence B. Afrin (2021). This article discusses the similarities between Mast Cell Activation Syndrome (MCAS) and Toxicant-Induced Loss of Tolerance (TILT), suggesting that both conditions share overlapping symptoms due to mast cell activation. It proposes that MCAS may provide a biological mechanism for TILT, with both conditions exhibiting multi-system symptoms triggered by environmental exposures. The article concludes that more research should be done to improve our understanding of the connection between TILT and MCs, and of new links between environmental exposures and illness.

Some cases of hypermobile Ehlers-Danlos syndrome may be rooted in mast cell activation syndrome

Lawrence B Afrin (2021).

Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association

Mast cell activation symptoms are prevalent in Long-COVID

Leonard B Weinstock, Jill B Brook, Arthur S Walters, Ashleigh Goris, Lawrence B Afrin, Gerhard J Molderings (2021).

COVID-19 infection in patients with mast cell disorders including mastocytosis does not impact mast cell activation symptoms

Matthew P Giannetti, Emily Weller, Iván Alvarez-Twose, Inés Torrado, Patrizia Bonadonna, Roberta Zanotti, Daniel F Dwyer, Dinah Foer, Cem Akin, Karin Hartmann, Tiago Azenha Rama, Wolfgang R Sperr, Peter Valent, Cristina Teodosio, Alberto Orfao, Mariana Castells (2021).

Mast Cell Activation Syndrome: A Primer for the Gastroenterologist

The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria

Zuberbier T, Abdul Latiff AH, Abuzakouk M, et al. The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria. Allergy. 2022;77:734–766. doi:10.1111/all.15090

Cosegregation of postural orthostatic tachycardia syndrome, hypermobile Ehlers-Danlos syndrome, and mast cell activation syndrome

Vadas P, Guzman J, McGillis L, Mittal N, Walsh S. Cosegregation of postural orthostatic tachycardia syndrome, hypermobile Ehlers-Danlos syndrome, and mast cell activation syndrome. Ann Allergy Asthma Immunol. 2020;125(6):719-720. doi:10.1016/j.anai.2020.08.015

The relationship between mast cell activation syndrome, postural tachycardia syndrome and Ehlers-Danlos syndrome

Wang E, Ganti T, Vaou E, Hohler A. The relationship between mast cell activation syndrome, postural tachycardia syndrome, and Ehlers-Danlos syndrome. Allergy Asthma Proc. 2021;42(3):243-246. doi:10.2500/aap.2021.42.210022

Mast cell activation syndromes and anaphylaxis: Multiple diseases part of the same spectrum

González-de-Olano D, Matito A, Alvarez-Twose I. Mast cell activation syndromes and anaphylaxis: Multiple diseases part of the same spectrum. Ann Allergy Asthma Immunol. 2020;124(2):143-145.e1. doi:10.1016/j.anai.2019.11.023

Patient characteristics of suspected mast-cell activation syndrome with sinonasal obstruction: a single institution experience

Kim JH, Xi S, Ference EH, Ge M, Liu MM, Wrobel BB. Patient characteristics of suspected mast-cell activation syndrome with sinonasal obstruction: a single institution experience. Int Forum Allergy Rhinol. 2020;10(8):996-1000. doi:10.1002/alr.22558

Depression, psychosocial correlates and psychosocial resources in individuals with mast cell activation syndrome.

Nicoloro SantaBarbara J, Lobel M. Depression, psychosocial correlates, and psychosocial resources in individuals with mast cell activation syndrome [published online ahead of print, 2021 May 18]. J Health Psychol. 2021;13591053211014583. doi:10.1177/13591053211014583

Mast cell activation syndrome as a significant comorbidity in sickle cell disease

Afrin LB. Mast cell activation syndrome as a significant comorbidity in sickle cell disease. Am J Med Sci. 2014;348(6):460-464. doi:10.1097/MAJ.0000000000000325

Mast cell activation syndrome in pain syndromes bladder pain syndrome/interstitial cystitis and vulvodynia

Regauer S. Mast cell activation syndrome in pain syndromes bladder pain syndrome/interstitial cystitis and vulvodynia. Transl Androl Urol. 2016;5(3):396-397. doi:10.21037/tau.2016.03.12

Restless legs syndrome is associated with mast cell activation syndrome

Weinstock LB, Walters AS, Brook JB, Kaleem Z, Afrin LB, Molderings GJ. Restless legs syndrome is associated with mast cell activation syndrome. J Clin Sleep Med. 2020;16(3):401-408. doi:10.5664/jcsm.8216

Comparing histamine intolerance and non-clonal mast cell activation syndrome.

Cimolai N. Comparing histamine intolerance and non-clonal mast cell activation syndrome. Intest Res. 2020;18(1):134-135. doi:10.5217/ir.2019.00087

The Relationship Between Hypermobile Ehlers-Danlos Syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS)

Kohn A, Chang C. The Relationship Between Hypermobile Ehlers-Danlos Syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS). Clin Rev Allergy Immunol. 2020;58(3):273-297. doi:10.1007/s12016-019-08755-8

Postural orthostatic tachycardia syndrome and the potential role of mast cell activation

Doherty TA, White AA. Postural orthostatic tachycardia syndrome and the potential role of mast cell activation. Auton Neurosci. 2018;215:83-88. doi:10.1016/j.autneu.2018.05.001

Risk of solid cancer in patients with mast cell activation

Molderings GJ, Zienkiewicz T, Homann J, Menzen M, Afrin LB. Risk of solid cancer in patients with mast cell activation syndrome: Results from Germany and USA. F1000Res. 2017;6:1889. Published 2017 Oct 26. doi:10.12688/f1000research.12730.1

Association of Postural Tachycardia Syndrome andEhlers-Danlos Syndrome with Mast Cell ActivationDisorders

Bonamichi-Santos R, Yoshimi-Kanamori K, Giavina-Bianchi P, Aun MV. Association of Postural Tachycardia Syndrome and Ehlers-Danlos Syndrome with Mast Cell Activation Disorders. Immunol Allergy Clin North Am. 2018;38(3):497-504. doi:10.1016/j.iac.2018.04.004

Mast cell disorders in Ehlers-Danlos syndrome

This report reviews basic biology of mast cells and mast cell activation as well as recent research efforts, which implicate a role of MC dysregulation beyond atopic disorders and in a cluster of Ehlers-Danlos Syndromes, non-IGE mediated hypersensitivity disorders, and dysautonomia.

Mast cell activation disease: An underappreciated cause of neurologic and psychiatric symptoms and diseases.

Afrin LB, Pöhlau D, Raithel M et al. Mast cell activation disease: An underappreciated cause of neurologic and psychiatric symptoms and diseases. Brain Behav Immun. 2015 Nov;50:314-21.

Mast cell activation syndromes presenting as anaphylaxis

3 Akin C . Mast cell activation syndromes presenting as anaphylaxis. Immunol Allergy Clin North Am. 2015 May;35(2):277-85.

A New Disease Cluster: Mast Cell Activation Syndrome, Postural Orthostatic Tachycardia Syndrome, and Ehlers-Danlos Syndrome

Cheung I, Vadas P. A New Disease Cluster: Mast Cell Activation Syndrome, Postural Orthostatic Tachycardia Syndrome, and Ehlers-Danlos Syndrome. Journal of Allergy and Clinical Immunology. Volume 135, Issue 2, AB65.

Mast cell activation disorders presenting with cerebral vasospasm-related symptoms: A “Kounis-like” syndrome

6 González-de-Olano D, Alvarez-Twose I, Matito A. Mast cell activation disorders presenting with cerebral vasospasm-related symptoms: A “Kounis-like” syndrome. Int J Cardiol. 2011 Jul 15;150(2):210-1.

New aspects of liver abnormalities as part of the systemic mast cell activation syndrome

Alfter K, von Kügelgen I, Haenisch B et al. New aspects of liver abnormalities as part of the systemic mast cell activation syndrome. Liver Int. 2009 Feb;29(2):181-6.

Hyperadrenergic Postural Tachycardia Syndrome in Mast Cell Activation Disorders

Shibao C, Arzubiaga C, Roberts LJ 2nd et al. Evidence of Mast Cell Activation Disorder in Postural Tachycardia Syndrome disorders. Hypertension. 2005 Mar;45(3):385-90.

Tests

Survey of Mast Cell Mediator Levels from Patients Presenting with Symptoms of Mast Cell Activation

Butterfield JH. Survey of Mast Cell Mediator Levels from Patients Presenting with Symptoms of Mast Cell Activation. Int Arch Allergy Immunol. 2020;181(1):43-50. doi:10.1159/000503964

Patients with mast cell activation symptoms and elevated baseline serum tryptase level have unique bone marrow morphology

Giannetti MP, Akin C, Hufdhi R, et al. Patients with mast cell activation symptoms and elevated baseline serum tryptase level have unique bone marrow morphology. J Allergy Clin Immunol. 2021;147(4):1497-1501.e1. doi:10.1016/j.jaci.2020.11.017

Distinct Small Intestine Mast Cell Histologic Changes in Patients With Hereditary Alpha-tryptasemia and Mast Cell Activation Syndrome

Hamilton MJ, Zhao M, Giannetti MP, et al. Distinct Small Intestine Mast Cell Histologic Changes in Patients With Hereditary Alpha-tryptasemia and Mast Cell Activation Syndrome. Am J Surg Pathol. 2021;45(7):997-1004. doi:10.1097/PAS.0000000000001676

A distinct biomolecular profile identifies monoclonal mast cell disorders in patients with idiopathic anaphylaxis.

Carter MC, Desai A, Komarow HD, et al. A distinct biomolecular profile identifies monoclonal mast cell disorders in patients with idiopathic anaphylaxis. J Allergy Clin Immunol. 2018;141(1):180-188.e3. doi:10.1016/j.jaci.2017.05.036

Highly sensitive assays are mandatory for the differential diagnosis of patients presenting with symptoms of mast cell activation: diagnostic workup of 38 patients

Van den Poel B, Kochuyt AM, Del Biondo E, et al. Highly sensitive assays are mandatory for the differential diagnosis of patients presenting with symptoms of mast cell activation: diagnostic work-up of 38 patients. Acta Clin Belg. 2017;72(2):123-129. doi:10.1080/17843286.2017.1293312

The Utility of Measuring Urinary Metabolites of Mast Cell Mediators in Systemic Mastocytosis and Mast Cell Activation Syndrome

Butterfield J, Weiler CR. The Utility of Measuring Urinary Metabolites of Mast Cell Mediators in Systemic Mastocytosis and Mast Cell Activation Syndrome. J Allergy Clin Immunol Pract. 2020;8(8):2533-2541. doi:10.1016/j.jaip.2020.02.021

Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome

Valent P, Bonadonna P, Hartmann K, 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(1):44-51. doi:10.1159/000501079

Determination of plasma heparin level improves identification of systemic mast cell activation disease.

Vysniauskaite M, Hertfelder HJ, Oldenburg J et al. Determination of plasma heparin level improves identification of systemic mast cell activation disease. PLoS One. 2015 Apr 24;10.4.

Prostaglandin D2-supplemented “functional eicosanoid testing and typing” assay with peripheral blood leukocytes as a new tool in the diagnosis of systemic mast cell activation disease: an explorative diagnostic study

Ravi A, Butterfield J, Weiler CR. Mast cell activation syndrome: improved identification by combined determinations of serum tryptase and 24-hour urine 11β-prostaglandin2α. J Allergy Clin Immunol Pract. 2014 Nov-Dec;2(6):775-8.

Mast cell activation syndrome: improved identification by combined determinations of serum tryptase and 24-hour urine 11β-prostaglandin2α.

Mast cell activation syndrome (MCAS) diagnosed using double-balloon enteroscopy

Mönkemüller K, Kassalik M, Baraksei D, Mast cell activation syndrome (MCAS) diagnosed using double-balloon enteroscopy. Endoscopy. 2012;44 Suppl 2 UCTN:E72-3

Genetics and Pathogenesis

KITD816V mutation in blood for the diagnostic screening of systemic mastocytosis and mast cell activation syndromes

Mast cell activation syndrome - anesthetic challenges in two different clinical scenarios

Idiopathic mast cell activation syndrome is more often suspected than diagnosed - A prospective real-life study

Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal

Pathogenic and diagnostic relevance of KIT in primary mast cell activation disorders

Muñoz-González JI, García-Montero AC, Orfao A, Álvarez-Twose I. PATHOGENIC AND DIAGNOSTIC RELEVANCE OF KIT IN PRIMARY MAST CELL ACTIVATION DISORDERS [published online ahead of print, 2021 Jul 20]. Ann Allergy Asthma Immunol. 2021;S1081-1206(21)00513-5. doi:10.1016/j.anai.2021.07.014

Effect of Dietary Fiber and Metabolites on Mast Cell Activation and Mast Cell-Associated Diseases

Folkerts J, Stadhouders R, Redegeld FA, et al. Effect of Dietary Fiber and Metabolites on Mast Cell Activation and Mast Cell-Associated Diseases. Front Immunol. 2018;9:1067. Published 2018 May 29. doi:10.3389/fimmu.2018.01067

Cellular Energetics of Mast Cell Development and Activation

Mendoza RP, Fudge DH, Brown JM. Cellular Energetics of Mast Cell Development and Activation. Cells. 2021;10(3):524. Published 2021 Mar 2. doi:10.3390/cells10030524

Mutational profiling in the peripheral blood leukocytes of patients with systemic mast cell activation syndrome using next-generation sequencing

Altmüller J, Haenisch B, Kawalia A, et al. Mutational profiling in the peripheral blood leukocytes of patients with systemic mast cell activation syndrome using next-generation sequencing. Immunogenetics. 2017;69(6):359-369. doi:10.1007/s00251-017-0981-y

The Circadian Clock Drives Mast Cell Functions in Allergic Reactions

Christ P, Sowa AS, Froy O, Lorentz A. The Circadian Clock Drives Mast Cell Functions in Allergic Reactions. Front Immunol. 2018;9:1526. Published 2018 Jul 6. doi:10.3389/fimmu.2018.01526

Epigenetic and transcriptional control of mast cell responses.

Monticelli S, Leoni C. Epigenetic and transcriptional control of mast cell responses. F1000Res. 2017;6:2064. Published 2017 Nov 29. doi:10.12688/f1000research.12384.1

Drug-Induced Pseudoallergy: A Review of the Causes and Mechanisms

Zhang B, Li Q, Shi C, Zhang X. Drug-Induced Pseudoallergy: A Review of the Causes and Mechanisms. Pharmacology. 2018;101(1-2):104-110. doi:10.1159/000479878

Mast Cell Activation Disease and Microbiotic Interactions

Afrin LB, Khoruts A. Mast Cell Activation Disease and Microbiotic Interactions. Clin Ther. 2015;37(5):941-953. doi:10.1016/j.clinthera.2015.02.008

Beyond IgE: Alternative Mast Cell Activation Across Different Disease States

Lyons DO, Pullen NA. Beyond IgE: Alternative Mast Cell Activation Across Different Disease States. Int J Mol Sci. 2020;21(4):1498. Published 2020 Feb 22. doi:10.3390/ijms21041498

Comparative analysis of mutation of tyrosine kinase kit in mast cells from patients with systemic mast cell activation syndrome and healthy subjects.

Molderings GJ, Meis K, Kolck UW et al. Comparative analysis of mutation of tyrosine kinase kit in mast cells from patients with systemic mast cell activation syndrome and healthy subjects. Immunogenetics. 2010 Dec;62(11-12):721-7.

NIH Video, scientists uncover genetic explanation for frustrating syndrome

Scientists at the National Institutes of Health have identified a genetic explanation for a syndrome characterized by multiple frustrating and difficult-to-treat symptoms.Most, but not all, people who experience these diverse symptoms have elevated levels of tryptase

Elevated basal serum tryptase identifies a multisystem disorder

JJ Lyons et al. Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number. Nature Genetics DOI: 10.1038/ng.3696 (2016).

Transgenerational transmission of systemic mast cell activation disease-genetic and epigenetic features

Molderings GJ. Transgenerational transmission of systemic mast cell activation disease-genetic and epigenetic features. Transl Res. 2016 Aug;174:86-97

The genetic basis of mast cell activation disease - looking through a glass darkly

Molderings GJ. The genetic basis of mast cell activation disease - looking through a glass darkly. Crit Rev Oncol Hematol. 2015 Feb;93(2):75-89.

Mendelian inheritance of elevated serum tryptase associated with atopy and connective tissue abnormalities

Lyons JJ, Sun G, Stone KD, et al. Mendelian inheritance of elevated serum tryptase associated with atopy and connective tissue abnormalities. J Allergy Clin Immunol. 2014 May;133(5):1471-4 .

Evidence for contribution of epigenetic mechanisms in the pathogenesis of systemic mast cell activation disease

Haenisch B, Fröhlich H, Herms S, Molderings GJ. Evidence for contribution of epigenetic mechanisms in the pathogenesis of systemic mast cell activation disease. Immunogenetics. 2014 May;66(5):287-97.

Familial Occurrence of Systemic Mast Cell Activation Disease

Molderings GJ, Haenisch B, Bogdanow M et al. Familial Occurrence of Systemic Mast Cell Activation Disease. PLoS One. 2013 Sep 30;8(9):e76241.

Systemic mast cell activation disease: the role of molecular genetic alterations in pathogenesis, heritability and diagnostics.

Haenisch B, Nöthen MM, Molderings GJ . Systemic mast cell activation disease: the role of molecular genetic alterations in pathogenesis, heritability and diagnostics. Immunology. 2012 Nov;137(3):197-205.

MCAS and COVID-19

Mast cell activation symptoms are prevalent in Long-COVID

Weinstock LB, Brook JB, Walters AS, Goris A, Afrin LB, Molderings GJ. Mast cell activation symptoms are prevalent in Long-COVID. Int J Infect Dis. 2021 Nov;112:217-226. doi: 10.1016/j.ijid.2021.09.043. Epub 2021 Sep 23. PMID: 34563706; PMCID: PMC8459548.

COVID-19 and Multisystem Inflammatory Syndrome, or is it Mast Cell Activation Syndrome?

Theoharides TC, Conti P. COVID-19 and Multisystem Inflammatory Syndrome, or is it Mast Cell Activation Syndrome?. J Biol Regul Homeost Agents. 2020;34(5):1633-1636. doi:10.23812/20-EDIT3

Risk and management of patients with mastocytosis and MCAS in the SARS-CoV-2 (COVID-19) pandemic: Expert opinions

o Valent P, Akin C, Bonadonna P, et al. Risk and management of patients with mastocytosis and MCAS in the SARS-CoV-2 (COVID-19) pandemic: Expert opinions. J Allergy Clin Immunol. 2020;146(2):300-306. doi:10.1016/j.jaci.2020.06.009

Potential association of mast cells with coronavirus disease 2019

Theoharides TC. Potential association of mast cells with coronavirus disease 2019. Ann Allergy Asthma Immunol. 2021;126(3):217-218. doi:10.1016/j.anai.2020.11.003

COVID-19 infection in patients with mast cell disorders including mastocytosis does not impact mast cell activation symptoms

o Giannetti MP, Weller E, Alvarez-Twose I, et al. COVID-19 infection in patients with mast cell disorders including mastocytosis does not impact mast cell activation symptoms. J Allergy Clin Immunol Pract. 2021;9(5):2083-2086. doi:10.1016/j.jaip.2021.02.023

Safety of COVID-19 vaccination in patients with mastocytosis and monoclonal mast cell activation syndrome

Kaakati R, Khokhar D, Akin C. Safety of COVID-19 vaccination in patients with mastocytosis and monoclonal mast cell activation syndrome [published online ahead of print, 2021 May 24]. J Allergy Clin Immunol Pract. 2021;S2213-2198(21)00588-2. doi:10.1016/j.jaip.2021.05.010

Persistent Antiphospholipid Antibodies, Mast Cell Activation Syndrome, Postural Orthostatic Tachycardia Syndrome and Post-COVID syndrome: 1 Year On

Schofield JR. Persistent Antiphospholipid Antibodies, Mast Cell Activation Syndrome, Postural Orthostatic Tachycardia Syndrome and Post-COVID Syndrome: 1 Year On. Eur J Case Rep Intern Med. 2021;8(3):002378. Published 2021 Mar 22. doi:10.12890/2021_002378

Covid-19 hyperinflammation and post-Covid-19 illness may be rooted in mast cell activation syndrome

Afrin LB, Weinstock LB, Molderings GJ. Covid-19 hyperinflammation and post-Covid-19 illness may be rooted in mast cell activation syndrome. Int J Infect Dis. 2020;100:327-332. doi:10.1016/j.ijid.2020.09.016

mRNA COVID-19 vaccine is well tolerated in patients with cutaneous and systemic mastocytosis with mast cell activation symptoms and anaphylaxis

Rama TA, Moreira A, Castells M. mRNA COVID-19 vaccine is well tolerated in patients with cutaneous and systemic mastocytosis with mast cell activation symptoms and anaphylaxis. J Allergy Clin Immunol. 2021;147(3):877-878. doi:10.1016/j.jaci.2021.01.004

MCAS and Medicines

Carbamazepine-induced Stevens-Johnson syndrome in a patient with history of methotrexate-induced mast cell activation syndrome

Zaalouk TM, Bitar ZI, Maadarani OS, Elhabibi ME. Carbamazepine-induced Stevens-Johnson syndrome in a patient with history of methotrexate-induced mast cell activation syndrome. Clin Case Rep. 2020;9(1):256-259. Published 2020 Nov 11. doi:10.1002/ccr3.3509

Adverse reactions to drugs and biologics in patients with clonal mast cell disorders: A Work Group Report of the Mast Cells Disorder Committee, American Academy of Allergy, Asthma & Immunology

Carter MC, Metcalfe DD, Matito A, et al. Adverse reactions to drugs and biologics in patients with clonal mast cell disorders: A Work Group Report of the Mast Cells Disorder Committee, American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol. 2019;143(3):880-893. doi:10.1016/j.jaci.2018.10.063

Safe administration of chemotherapy in mast cell activation syndrome

Lythgoe MP, Krell J, McNeish IA, Tookman L. Safe administration of chemotherapy in mast cell activation syndrome. J Oncol Pharm Pract. 2021;27(4):1005-1010. doi:10.1177/1078155220953879

Recognition and Management of Medication Excipient Reactivity in Patients With Mast Cell Activation Syndrome

Schofield JR, Afrin LB. Recognition and Management of Medication Excipient Reactivity in Patients With Mast Cell Activation Syndrome. Am J Med Sci. 2019;357(6):507-511. doi:10.1016/j.amjms.2019.03.005

Safety of COVID-19 vaccination in patients with mastocytosis and monoclonal mast cell activation syndrome

mRNA COVID-19 vaccine is well tolerated in patients with cutaneous and systemic mastocytosis with mast cell activation symptoms and anaphylaxis

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