• J. Allergy Clin. Immunol. · Jul 2015

    Clonal mast cell disorders in patients with severe Hymenoptera venom allergy and normal serum tryptase levels.

    • Roberta Zanotti, Carla Lombardo, Giovanni Passalacqua, Cristian Caimmi, Massimiliano Bonifacio, Giovanna De Matteis, Omar Perbellini, Maurizio Rossini, Donatella Schena, Moira Busa, Maria Cinzia Marcotulli, Maria Beatrice Bilò, Maurizio Franchini, Giovanni Marchi, Livio Simioni, and Patrizia Bonadonna.
    • Department of Medicine, Section of Hematology, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy; Multidisciplinary Mastocytosis Outpatient Clinic, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
    • J. Allergy Clin. Immunol. 2015 Jul 1; 136 (1): 135-9.

    BackgroundSystemic mastocytosis is a clonal mast cell (MC) disease that can lead to potentially fatal anaphylactic reactions caused by excessive MC mediator release. The prevalence of mastocytosis in patients with Hymenoptera venom allergy is high, and thus the disease should be suspected in patients with severe reactions caused by Hymenoptera stings and increased serum basal tryptase (SBT) levels.ObjectiveWe sought to evaluate the presence of clonal MC disorders in patients seen at our mastocytosis center with Hymenoptera sting-induced anaphylaxis, documented hypotension, absence of urticaria pigmentosa, and normal SBT levels.MethodsTwenty-two patients with Hymenoptera sting-induced anaphylaxis, without skin lesions, and with tryptase levels of less than 11.4 ng/mL underwent bone marrow evaluation. Bone mineral density was assessed in those patients with ascertained mastocytosis.ResultsIn 16 of 22 patients, a diagnosis of indolent mastocytosis could be established, and 1 patient had a monoclonal MC activation syndrome. Patients with mastocytosis had higher SBT levels (P = .03) but only rarely had angioedema/urticaria associated with hypotension (P = .004).ConclusionsThe absence of urticaria or angioedema in severe reactions to Hymenoptera stings with hypotension might represent the most relevant factor in identifying patients with mastocytosis, regardless of their serum tryptase levels.Copyright © 2015 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

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