Successful venom immunotherapy with avapritinib in a patient with systemic mastocytosis
Systemic mastocytosis (SM) is a clonal mast cell (MC) disorder characterized by tissue infiltration of MCs driving clinical symptoms.1 The disorder is characterized by activating mutations in the KIT protein, classically KIT p.D816V. SM is treated in a symptom-guided manner with therapies targeted toward bioactive MC mediators. However, the recent discoveries of targeted tyrosine kinase inhibitors have changed the treatment paradigm of indolent SM (ISM).2 Tyrosine kinase inhibitors have demonstrated to reduce MC burden and improve quality of life. To date, they have not been used to facilitate venom immunotherapy (VIT). Patients with mastocytosis and Hymenoptera venom allergy may have recurrent anaphylaxis during VIT, and adjunctive therapies are a significant unmet need to protect this patient population. Although omalizumab has been found to be effective in a subset of patients, non-IgE-mediated mechanisms may be activated by Hymenoptera venom and an increased mutated MC burden may contribute to life-threatening symptoms. We report, here, the first case of successful VIT in a patient with ISM treated with avapritinib.
Depina, L., Karmarkar, S. N., Moeller, S., Lazarovich, M., De Magalhães, A. R., Castells, M., & Giannetti, M. P. (2025). Successful venom immunotherapy with avapritinib in a patient with systemic mastocytosis.
Annals of Allergy, Asthma & Immunology,
135(3), 343-344.
https://doi.org/10.1016/j.anai.2025.06.015
