Hymenoptera Allergy and Mast Cell Activation Syndromes
- 615 Downloads
Mast cell activation syndrome (MCAS) can be diagnosed in patients with recurrent, severe symptoms from mast cell (MC)-derived mediators, which are transiently increased in serum and are attenuated by mediator-targeting drugs. When KIT-mutated, clonal MC are detected in these patients, a diagnosis of primary MCAS can be made. Severe systemic reactions to hymenoptera venom (HV) represent the most common form of anaphylaxis in patients with mastocytosis. Patients with primary MCAS and HV anaphylaxis are predominantly males and do not have skin lesions in the majority of cases, and anaphylaxis is characterized by hypotension and syncope in the absence of urticaria and angioedema. A normal value of tryptase (≤11.4 ng/ml) in these patients does not exclude a diagnosis of mastocytosis. Patients with primary MCAS and HV anaphylaxis have to undergo lifelong venom immunotherapy, in order to prevent further potentially fatal severe reactions.
KeywordsSystemic mastocytosis Mast cell activation syndromes Hymenoptera venom allergy Anaphylaxis Tryptase Clonal mast cell activation syndromes
Compliance with Ethical Standards
Conflict of Interest
Dr. Bonifacio reports grants from Novartis Farma and personal fees from Ariad Pharmaceuticals and Amgen. Drs. Bonadonna, Bonifacio, Lombardo, and Zanotti declare no conflicts 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.
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. 2012;157:215–25. Consensus paper from a group of experts defining criteria for diagnosis of mast cell disorders and proposing a global unifying classification of all mast cell disorders. These criteria help to diagnose mast cell activation syndromes.PubMedCentralCrossRefPubMedGoogle Scholar
- 3.•Niedoszytko M, Bonadonna P, Oude Elberink JN, Golden DB. Epidemiology, diagnosis, and treatment of Hymenoptera venom allergy in mastocytosis patients. Immunol Allergy Clin North Am. 2014;34(2):365–81. Complete review about the management of patients with clonal mast cell disorders and Hymenoptera venom allergy, with particular attention of diagnosis and treatment of these patients.CrossRefPubMedGoogle Scholar
- 6.Horny HP et al., editors. WHO classification of tumors of hematopoietic and lymphoid tissues. 4th ed. Lyon (France): IARC Press; 2008. p. 54–63.Google Scholar
- 7.Garcia-Montero AC, Jara-Acevedo M, Teodosio C, Sanchez ML, Nunez R, Prados A, et al. A 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):236672.CrossRefGoogle Scholar
- 10.Alvarez-Twose I, Gonzalez de Olano D, Sánchez-Muñoz L, Matito A, Esteban-López MI, Vega A, et al. Clinical, biological and molecular characteristics of systemic mast cell disorders presenting with severe mediator-related symptoms. J Allergy Clin Immunol. 2010;125:1269–78.CrossRefPubMedGoogle Scholar
- 11.Alvarez-Twose I, Zanotti R, González-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;133:520–8.CrossRefPubMedGoogle Scholar
- 20.Gonzalez de Olano D, Hoz de la Caballer B, Núñez López R, Sánchez Muñoz L, Cuevas Agustín M, Diéguez 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;37:1547–55.PubMedGoogle Scholar
- 27.Gonzalez-de-Olano D, Alvarez-Twose I, Esteban-López MI, Sánchez-Muñoz 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;121:519–26.CrossRefPubMedGoogle Scholar
- 31.••Zanotti R, Lombardo C, Passalacqua G, Caimmi C, Bonifacio M, De Matteis G, et al. Clonal mast cell disorders in patients with severe Hymenoptera venom allergy and normal serum tryptase levels. J Allergy Clin Immunol. 2015;136(1):135–9. Paper that shows that the absence of urticaria or angioedema in severe reactions to hymenoptera stings with hypotension may represent the most relevant factor in identifying patients with mastocytosis regardless of their serum tryptase.CrossRefPubMedGoogle Scholar
- 49.Incorvaia C, Mauro M, Pravettoni V, Pucci S. Hypersensitivity to Hymenoptera venom: advances in diagnosis and implications for treatment. Recent Patents on Inflammation & Allergy Drug Discovery 2011, 5, 128–135 (A)Google Scholar
- 58.Mosbech H, Christensen J, Dirksen A, Soborg M. Insect allergy. Predictive value of diagnostic tests: a three-year follow-up study. Clin Allergy 1986;16:433–40.25 venom immunotherapyGoogle Scholar
- 60.Ridolo E, Montagni M, Bonzano L, Savi E, Peveri S, Costantino MT, et al. How far from correct is the use of adrenaline auto-injectors? A survey in Italian patients. Emerg Med. 2015Google Scholar