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.

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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