Mast Cell Disorders: Overview, Clinical Pearls and Perspectives

Authors

  • Luis Murguia-Favela, MD, FRCPC

DOI:

https://doi.org/10.58931/cait.2021.1217

Abstract

It has been almost 145 years since the discovery of mast cells (MC) and we continue to learn about their function and the consequences of their dysregulation. The focus of this article is to provide an overview on the current, yet evolving, classification of mast cell disorders (MCD) and provide clinical pearls and perspectives for their management for the allergist/ immunologist (A/I).

Author Biography

Luis Murguia-Favela, MD, FRCPC

Dr. Murguia-Favela is a pediatric immunologist at the Alberta Children’s Hospital and a Clinical Associate Professor at the University of Calgary. He earned his medical degree from the National Autonomous University of Mexico and then re-trained in pediatrics and clinical immunology and allergy at the University of Toronto. As a researcher for the Alberta Children’s Hospital Research Institute, his interests are mainly focused on primary immune deficiencies and immune dysregulation disorders. He is the site co-investigator for the Primary Immune Deficiency Treatment Consortium (PIDTC), and a founding member and current chair of the Clinical Immunology Network-Canada (CINC), a pan-Canadian group of clinical immunologists focused around generating and collaborating on clinical research, education, and advocacy projects in the field of Immunology.

References

da Silva EZ, Jamur MC, Oliver C. Mast cell function: a new vision of an old cell. J Histochem Cytochem. 2014 Oct;62(10):698-738. doi:10.1369/0022155414545334

Varricchi G, de Paulis A, Marone G, Galli SJ. Future needs in mast cell biology. Int. J. Mol. Sci. 2019 Sep 6;20(18):4397.

Galli SJ, Kalesnikoff J, Grimbaldeston MA, Piliponsky AM, Williams CMM, Tsai M. Mast cells as “tunable”effector and immunoregulatory cells: recent advances. Annu. Rev. Immunol. 2005;23:749-86. doi:10.1146/annurev.immunol.21.120601.141025

Hendriksen E, van Bergeijk D, Oosting RS, Redegeld FA. Mast cells in neuroinflammation and brain disorders. Neurosci. Biobehav. Rev. 2017 Aug;79:119-133. doi:10.1016/j.neubiorev.2017.05.001

Gilfillan AM, Austin SJ, Metcalfe DD. Mast cell biology: introduction and overview. Adv Exp Med Biol. 2011;716:2-12. doi:10.1007/978-1-4419-9533-9_1

Theoharides TC, Valent P, Akin C. Mast cells, mastocytosis, and related disorders. N Engl J Med. 2015 Jul 9;373(2):163-72. doi:10.1056/NEJMra1409760

Siraganian RP. Mast cell signal transduction from the high-affinity IgE receptor. Curr Opin Immunol. 2003 Dec;15(6):639-46. doi:10.1016/j.coi.2003.09.010

Mukai K, Tsai M, Saito H, Galli SJ. Mast cells as sources of cytokines, chemokines and growth factors. Immunol Rev. 2018 March;282(1):121-150. doi:10.1111/imr.12634

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 of Clin Immunol. 2019 Jun;15(6):639-656. doi:10.1080/1744666X.2019.1596800

Panula P, Chazot PL, Cowart M, Gutzmer R, Leurs R, Liu WLS, Stark H, Thurmond RL, Haas HL. International Union of Basic and Clinical Pharmacology. XCVIII. Histamine receptors. Pharmacol Rev. 2015 Jul;67(3):601-655. doi:10.1124/pr.114.010249

Zhang T, Finn DF, Barlow JW, Walsh JJ. Mast cell stabilisers. Eur J Pharmacol. 2016 May 5;778:158-168. doi:10.1016/j.ejphar.2015.05.071.

Gülen T, Akin C. Pharmacotherapy of mast cell disorders. Curr Opin Allergy Clin Immunol. 2017 Aug;17(4):295-303. doi:10.1097/ACI.0000000000000377

Valent P, Akin C, Arock M, Brockow K, Butterfield JH, Carter MC, 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;157:215-225. doi:10.1159/000328760

Valent P. Mast cell activation syndromes: definition and classification. Allergy. 2013 Apr; 68(4):417-24. doi: 10.1111/all.12126.

Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, et al. Cutaneous manifestations in patients with mastocytosis: consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol 2016 Jan;137(1):35-45. doi:10.1016/j.jaci.2015.08.034

Wilcock A, Bahri R, Bulfone-Paus S, Arkwright PD. Mast cell disorders: from infancy to maturity. Allergy. 2019 Jan;74(1):53-63. doi:10.1111/all.13657

Galen BT, Rose MG. Darier’s sign in mastocytosis. Blood. 2014 Feb 20;123(8):1127. doi:10.1182/blood-2013-11-538355

Klaiber N, Kumar S, Irani AM. Mastocytosis in children. Curr Allergy Asthma Rep. 2017 Oct 7;17(11):80. doi:10.1007/s11882-017-0748-4

Pardanani A. Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management. Am J Hematol. 2019 mar;94(3):363-377. doi:10.1002/ajh.25371

Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016;127:2391-2405. doi:10.1182/blood-2016-03-643544

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 May;35(5):548-562. doi:10.1016/j.clinthera.2013.04.001

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

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 Jun;58(3):273-297. doi:10.1007/s12016-019-08755-8

Seneviratne SL, Maitland A, Afrin L. Mast cell disorders in Ehlers-Danlos syndrome. Am J Med Genet Part C Semin Med Genet. 2017 Mar;175(1):226-236. doi:10.1002/ajmg.c.31555

Conti P, Gallenga CE, Ronconi G, et al. Activation of mast cells mediates inflammatory response in psoriasis: potential new therapeutic approach with IL-37. Dermatol Ther. 2019 Jul;32(4):e12943. doi:10.1111/dth.12943

Theoharides TC, Kempuraj D, Sant GR. Mast cell involvement in interstitial cystitis: a review of human and experimental evidence. Urology. 2001 Jun;57(6 Suppl 1):47-55. doi:10.1016/s0090-4295(01)01129-3

Hsieh FH. Gastrointestinal involvement in mast cell activation syndromes. Immunol Allergy Clin North Am. 2018 Aug;38(3):429-441. doi:10.1016/j.iac.2018.04.008

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

Jennings SV, Slee VM, Zack RM, Verstovsek S, George TI, Shi H, et al. Patient perceptions in mast cell disorders. Immunol Allergy Clin North Am. 2018 Aug;38(3):505-525. doi:10.1016/j.iac.2018.04.006

Mast Cell Disorders: Overview, Clinical Pearls and Perspectives

Published

2021-06-01

How to Cite

1.
Murguia-Favela L. Mast Cell Disorders: Overview, Clinical Pearls and Perspectives. Can Allergy Immunol Today [Internet]. 2021 Jun. 1 [cited 2024 Nov. 24];1(2):6–15. Available from: https://canadianallergyandimmunologytoday.com/article/view/vol-1-2-murguia-favela

Issue

Section

Articles