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

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.