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Curr Allergy Asthma Rep. 2016 Jan;16(1):5. doi: 10.1007/s11882-015-0582-5.

Hymenoptera Allergy and Mast Cell Activation Syndromes.

Author information

1
Allergy Unit, Azienda Ospedaliera Universitaria Integrata di Verona, Piazzale Stefani 1, 37126, Verona, Italy. patrizia.bonadonna@ospedaleuniverona.it.
2
Multidisciplinary Outpatients clinics for Mastocytosis (GISM), Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy. patrizia.bonadonna@ospedaleuniverona.it.
3
Section of Hematology, Department of Medicine, Azienda Ospedaliera Universitaria Integrata di Verona, Piazzale L.A. Scuro, 10-37134, Verona, Italy. massimiliano.bonifacio@univr.it.
4
Multidisciplinary Outpatients clinics for Mastocytosis (GISM), Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy. massimiliano.bonifacio@univr.it.
5
Allergy Unit, Azienda Ospedaliera Universitaria Integrata di Verona, Piazzale Stefani 1, 37126, Verona, Italy. carla.lombardo@ospedaleuniverona.it.
6
Multidisciplinary Outpatients clinics for Mastocytosis (GISM), Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy. carla.lombardo@ospedaleuniverona.it.
7
Section of Hematology, Department of Medicine, Azienda Ospedaliera Universitaria Integrata di Verona, Piazzale L.A. Scuro, 10-37134, Verona, Italy. roberta.zanotti@univr.it.
8
Multidisciplinary Outpatients clinics for Mastocytosis (GISM), Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy. roberta.zanotti@univr.it.

Abstract

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.

KEYWORDS:

Anaphylaxis; Clonal mast cell activation syndromes; Hymenoptera venom allergy; Mast cell activation syndromes; Systemic mastocytosis; Tryptase

PMID:
26714690
DOI:
10.1007/s11882-015-0582-5
[Indexed for MEDLINE]

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