Anaphylaxis in Mastocytosis*

M.C. Castells (ed.), Anaphylaxis and Hypersensitivity Reactions, DOI 10.1007/978-1-60327-951-2_16, © Springer Science+Business Media, LLC 2011 Abstract An increase in anaphylaxis has been reported in mastocytosis, with a predominance of males. Recurrent idiopathic anaphylaxis and either hymenoptera-venom or drug-induced anaphylaxis are the most frequent types of anaphylaxis, no correlation being found between the signs and symptoms of anaphylaxis and MC burden or serum tryptase levels. Less frequently, anaphylaxis can occur during general anesthesia, and other invasive and therapeutic procedures, indicating that appropriate management of SM patients before, during, and after these procedures is required. Venom immunotherapy (VIT) has proved to be effective in mastocytosis, but adverse reactions have also been reported in 10–15% of the patients and fatal reactions have been described after discontinuation of venom immunotherapy. Therapeutic measures of anaphylaxis in mastocytosis should include: (1) adequate information and training of patients, their relatives, and care providers, (2) availability of individual emergency kits, (3) avoidance of triggering factors, and (4) administration of preventive medication prior to anesthesia and other therapeutic procedures. Additionally, specific treatment of anaphylaxis should be carefully evaluated in each individual patient. Prompt recognition and appropriate control of the acute episodes together with a decrease in the frequency and intensity of chronic symptoms should be attempted with antimediator therapy (e.g. sedating and nonsedating H1 antihistamines and other NSAIDs, oral disodium cromolyn or, in selected cases, aspirin). Steroids should be used in selected cases. Interferon-alpha, hydroxyurea, cytoreductive therapy with cladribine, and omalizumab anti-IgE monoclonal antibody therapy might be of benefit in selected refractory cases.