Mast Cell Disorders and Hymenoptera Venom-Triggered Anaphylaxis: Evaluation and Management.

Patients with Hymenoptera venom allergy (HVA), especially those with severe anaphylaxis, frequently have concomitant clonal mast cell disease (MCD) in the form of systemic mastocytosis or monoclonal mast cell activation syndrome. Detection of clonal MCD is important because it will have significant consequences for managing HVA. Therefore, we recommend patients with HVA be systematically screened for clonal MCD. The pretest probability of clonal MCD can be assessed in a stepwise fashion starting with examination of the skin for typical monomorphic maculopapular cutaneous mastocytosis lesions; measurement of the baseline serum tryptase (BST) and tryptase genotyping for patients with BST greater than 11 ng/mL; followed by the Red Española de Mastocitosis score, which is calculated using anaphylaxis clinical features, BST, and the patient's sex. A bone marrow biopsy should be performed in patients with monomorphic maculopapular cutaneous mastocytosis, a Red Española de Mastocitosis score of 2 or greater, or an elevated BST based on tryptase genotype. Patients with HVA and a clonal MCD should be treated with immunotherapy directed against the Hymenoptera venom for which they are sensitized. For this high-risk subgroup of patients with HVA, it is recommended to continue immunotherapy for more than 5 years or indefinitely and to carry at least three epinephrine autoinjectors. Future studies should determine whether KIT D816V-selective tyrosine kinase inhibitors are effective at preventing or reducing the severity of Hymenoptera-venom triggered anaphylaxis in patients with clonal MCD.