Anaphylaxis Research Today is a free monthly online journal that collates and summarizes the latest research about Anaphylaxis, including details on food allergies, diagnosis, treatment, causes.

Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients.

Brockow K, Jofer C, Behrendt H, Ring J

Department of Dermatology and Allergy Biederstein, Technical University Munich, Munich, Germany.

BACKGROUND: Excessive mast cell mediator release may lead to anaphylaxis in patients with mastocytosis. However, the incidence, clinical features and trigger factors have not yet been analyzed. METHODS: To identify risk factors for anaphylaxis in mastocytosis, we determined cumulative incidence, severity, clinical characteristics, and trigger factors for anaphylaxis in 120 consecutive patients (53 male; 67 female, median age and range 24 years, 1 month to 73 years), and correlated these with disease severity of mastocytosis, skin involvement, basal total serum tryptase, and diaminooxidase concentrations. RESULTS: The cumulative incidence of anaphylaxis in patients with mastocytosis was higher in adults (49%; P < 0.01) compared with that in children (9%). Only children with extensive skin involvement had experienced anaphylaxis. In adults, anaphylaxis was correlated to the absence of urticaria pigmentosa lesions (P < 0.03). Reactions occurred more frequently in adults with systemic (56%) when compared with cutaneous mastocytosis (13%; P < 0.02). In adults, 48% of reactions were severe, and 38% resulted in unconsciousness. Major perceived trigger factors for adults were hymenoptera stings (19%), foods (16%), and medication (9%); however, in 26% of reactions, only a combination of different triggers preceded anaphylaxis. Trigger factors remained unidentified in 67% of reactions in children compared with 13% in adults. Patients with anaphylaxis had higher basal tryptase values (60.2 +/- 55 ng/ml, P < 0.0001) in comparison with those without (21.2 +/- 33 ng/ml), but not diaminooxidase levels. CONCLUSION: Adult patients and children with extensive skin disease with mastocytosis have an increased risk to develop severe anaphylaxis; thus, an emergency set of medication including epinephrine is recommended.

Published 11 January 2008 in Allergy, 63(2): 226-32.
Full-text of this article is available online (may require subscription).

© 2005-2008 Anaphylaxis Research Today. All Rights Reserved.