Abstract
Up to 5% of adult patients with Hymenoptera venom allergy (HVA) may simultaneously suffer from a clonal mast cell disease. Usually, a mastocytosis or a monoclonal mast cell activation syndrome is not diagnosed before the appearance of a sting anaphylaxis. The majority of patients with a clonal mast cell disease and HVA will not present with significant symptoms except osteoporosis and symptoms of mast cell release due to other triggers. The diagnosis of a mast cell disease will lead to several therapeutic consequences concerning the treatment of HVA. Therefore, it is recommended to screen all adults with systemic sting reactions for an increased baseline tryptase concentration (BTC, >11.4 μg/l) and mastocytosis in the skin (MIS). Patients with MIS, a significantly increased BTC (>20 μg/l), and/or a history of a severe anaphylactic sting reaction (i.e., anaphylactic shock or near-fatal reactions) require further examination. If MIS is absent or if it is necessary to identify the exact type of clonal mast cell disease, patient work-up should include a liquid biopsy (i.e., screening for a KIT D816V mutation in the peripheral blood) and a bone marrow biopsy. Venom immunotherapy (VIT) is urgently recommended for patients with a clonal mast cell disease and should include an increased maintenance dose right from the beginning in patients with a honeybee venom allergy or with a history of near-fatal sting reactions. Patients with a clonal mast cell disease are at an increased risk for treatment failure; therefore, we suggest to perform sting challenge tests in order to document the efficacy of VIT. VIT should be continued lifelong.
Published Version
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