Hymenoptera Allergy and Mast Cell Activation Syndromes
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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
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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.
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This article does not contain any studies with human or animal subjects performed by any of the authors.
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