Clonal mast cell disorders (cMCD) comprise systemic mastocytosis (SM) and monoclonal mast cell activation syndrome (MMAS). 1,2 Common to these two conditions is the presence of mast cell (MC) clonality, as reflected in a mutation in codon 816 of KIT and/or occurrence of immunophenotypically aberrant MCs expressing CD25. 3 In patients with MMAS, the WHO criteria for SM are not fully met. 3 Anaphylaxis is a well-known feature of cMCD; particularly, venom allergy represents an increased risk of severe, even fatal, sting anaphylaxis in these patients. 4,5 Although the overall prevalence of Hymenoptera venom-induced anaphylaxis (HVA) is approximately 25% in patients with SM, 6 the underlying reason(s) for this association remains elusive. The aggravated risk of severe HVA might be due to increased MC burden, perivascular aggregation of MCs and an amplified IgE reaction due to the presence of D816V KIT mutation. 7 These findings stress the importance of
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