Clonal mast cell disorders in patients with severe Hymenoptera venom allergy and normal serum tryptase levels - 03/07/15
, Cristian Caimmi, MD e, Massimiliano Bonifacio, MD a, b, Giovanna De Matteis, MD f, Omar Perbellini, MD a, Maurizio Rossini, MD b, e, Donatella Schena, MD b, g, Moira Busa, MD h, Maria Cinzia Marcotulli, MD i, Maria Beatrice Bilò, MD j, Maurizio Franchini, MD k, Giovanni Marchi, MD l, Livio Simioni, MD m, Patrizia Bonadonna, MD b, cAbstract |
Background |
Systemic 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.
Objective |
We 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.
Methods |
Twenty-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.
Results |
In 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).
Conclusions |
The 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.
Le texte complet de cet article est disponible en PDF.Key words : Systemic mastocytosis, anaphylaxis, tryptase, Hymenoptera venom allergy
Abbreviations used : BM, CMD, HVA, IQR, ISM, MC, ROC, SBT, SM
Plan
| Disclosure of potential conflict of interest: The authors declare that they have no relevant conflicts of interest. |
Vol 136 - N° 1
P. 135-139 - juillet 2015 Retour au numéro
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