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:
Mast cell activation disease and immunoglobulin deficiency in patients with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorder (November 2021)
- Some cases of hypermobile Ehlers-Danlos syndrome may be rooted in mast cell activation syndrome (October 2021)
Mast cell activation symptoms are prevalent in Long-COVID (September 2021)
- Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association (August 2021)
Safe administration of chemotherapy in mast cell activation syndrome (June 2021)
Diagnosis
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
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
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
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.
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 (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
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
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
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
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