Mast Cell Activation Syndromes and Environmental Exposures
- 13 Downloads
Mast cell activation syndromes (MCAS) are an emerging entity that includes a heterogeneous group of diseases presenting with typical signs and symptoms secondary to the release of mast cell (MC) mediators. Triggering factors underlying the activation of mast cells show a wide variability, and episodes of MC-mediator release are usually triggered by idiopathic or by mixed causes, which makes avoidance recommendations difficult to devise. Sometimes, environmental or occupational exposures are responsible for MC activation. In this article, we review the main workplace environment eliciting factors described in MCAS and the current guidance on treatment.
KeywordsClonal Environmental Mast cell Mast cell activation syndrome Mastocytosis Occupational
We thank Isabel Coman for her technical assistance with the revision of the English version of the manuscript.
Compliance with ethical standards
Conflict of interest
David González-de-Olano declares that he has no conflict of interest. Javier Domínguez-Ortega declares that he has no conflict of interest. Silvia Sánchez-García declares that she has no conflict of interest.
Human and animal rights and informed consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 1.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. 2011;157(3):215–25.CrossRefPubMedPubMedCentralGoogle Scholar
- 2.Akin C, Valent P, Metcalfe DD. Mast cell activation syndrome: Proposed diagnostic criteria. J Allergy Clin Immunol. 2010;126(6):1099–104. e1094Google Scholar
- 14.• Gonzalez-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. Complete and easy to read review of mast cell activation syndromes and their current recommended management.CrossRefPubMedPubMedCentralGoogle Scholar
- 15.•• Alvarez-Twose I, Gonzalez de Olano D, Sanchez-Munoz L, Matito A, Esteban-Lopez MI, Vega A, et al. Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms. J Allergy Clin Immunol. 2010;125(6):1269–78. e1262. This is the first article that describes the clinical and biological characteristics of the clonal mast cell disorders, and helps differenciate with other types of MCD. Besides, a helpful screening tool that suggests clonality is provided.CrossRefPubMedGoogle Scholar
- 16.Alvarez-Twose I, Zanotti R, Gonzalez-de-Olano D, Bonadonna P, Vega A, Matito A, et al. Nonaggressive systemic mastocytosis (sm) without skin lesions associated with insect-induced anaphylaxis shows unique features versus other indolent sm. J Allergy Clin Immunol. 2014;133(2):520–8.CrossRefPubMedGoogle Scholar
- 23.Garcia-Montero AC, Jara-Acevedo M, Teodosio C, Sanchez ML, Nunez R, Prados A, et al. Kit mutation in mast cells and other bone marrow hematopoietic cell lineages in systemic mast cell disorders: A prospective study of the spanish network on mastocytosis (rema) in a series of 113 patients. Blood. 2006;108(7):2366–72.CrossRefPubMedGoogle Scholar
- 25.Alvarez-Twose I, Gonzalez-de-Olano D, Sanchez-Munoz L, Matito A, Jara-Acevedo M, Teodosio C, et al. Validation of the rema score for predicting mast cell clonality and systemic mastocytosis in patients with systemic mast cell activation symptoms. Int Arch Allergy Immunol. 2011;157(3):275–80.CrossRefPubMedGoogle Scholar
- 28.Alvarez-Twose I, Matito A, Sanchez-Muñoz L, Morgado JM, Escribano L. Management of adult mastocytosis. Expert Opin Orphan Drugs. 2014;2(4):16.Google Scholar
- 32.•• Siracusa A, Folletti I, Gerth van Wijk R, Jeebhay MF, Moscato G, Quirce S, et al. Occupational anaphylaxis--an eaaci task force consensus statement. Allergy. 2015;70(2):141–52. This is a complete document assessing the main causes of anaphylaxis at the work place. If we recognized a possible occupation at risk of anaphylaxis, we could identify a potential risk for severe systemic reactions in a worker with mastocytosis.CrossRefPubMedGoogle Scholar
- 33.•• Gulen T, Ljung C, Nilsson G, Akin C. Risk factor analysis of anaphylactic reactions in patients with systemic mastocytosis. J Allergy Clin Immunol Pract. 2017;5(5):1248–55. This article provides a risk analysis model, offering a simple method of identifying patients with mastocytosis at high risk of anaphylaxis.CrossRefPubMedGoogle Scholar
- 36.Bonadonna P, Lombardo C, Zanotti R. Mastocytosis and allergic diseases. J Investig Allergol Clin Immunol. 2014;24(5):288–97. quiz 283 p preceding 297Google Scholar
- 37.Gonzalez de Olano D, de la Hoz Caballer B, Nunez Lopez R, Sanchez Munoz L, Cuevas Agustin M, Dieguez MC, et al. Prevalence of allergy and anaphylactic symptoms in 210 adult and pediatric patients with mastocytosis in spain: A study of the spanish network on mastocytosis (rema). Clin Exp Allergy. 2007;37(10):1547–55.PubMedGoogle Scholar
- 49.• Morales-Cabeza C, Prieto-Garcia A, Acero S, Bartolome-Zavala B, Morgado JM, Matito A, et al. Systemic mastocytosis presenting as occupational ige-mediated anaphylaxis to pine processionary caterpillar. Ann Allergy Asthma Immunol. 2016;117(3):333–4. e331. A very well-described case of occupational anaphylaxis due to the contact to an insect exclusively at the work place, and the ulterior allergologic work-up.CrossRefPubMedGoogle Scholar
- 53.Broesby-Olsen S, Vestergaard H, Mortz CG, Jensen B, Havelund T, Hermann AP, et al. Omalizumab prevents anaphylaxis and improves symptoms in systemic mastocytosis: Efficacy and safety observations. Allergy. 2017. https://doi.org/10.1111/all.13237.
- 54.Antolin-Amerigo D, Ruiz-Leon B, Boni E, Alfaya-Arias T, Alvarez-Mon M, Barbarroja-Escudero J, et al. Component-resolved diagnosis in hymenoptera allergy. Allergol Immunopathol (Madr). 2017. https://doi.org/10.1016/j.aller.2017.05.003.
- 57.Gonzalez de Olano D, Alvarez-Twose I, Esteban-Lopez MI, Sanchez-Munoz L, de Durana MD, Vega A, et al. Safety and effectiveness of immunotherapy in patients with indolent systemic mastocytosis presenting with hymenoptera venom anaphylaxis. J Allergy Clin Immunol. 2008;121(2):519–26.CrossRefPubMedGoogle Scholar