20.500.12556/RUL-164839 Fatal Hymenoptera venom–triggered anaphylaxis in patients with unrecognized clonal mast cell disorder is mastocytosis to blame? Hymenoptera venom–triggered anaphylaxis (HVA) affects up to 8.9% of the general population and is the most frequent cause of anaphylaxis in adults, accounting for approximately 20% of all fatal anaphylaxis cases. Quite often, a fatal reaction is a victim’s first manifestation of HVA. Mastocytosis represents one of the most important risk factors for severe HVA. We analyzed patients with documented fatal HVA for the presence of underlying clonal mast cell disorder (cMCD). Here, we report three cases of fatal HVA, with undiagnosed underlying cMCD identified by the presence of the peripheral blood and/or bone marrow KIT p.D816V missense variant postmortem. In the first case, anaphylaxis was the initial episode and was fatal. In the other two cases, both patients were treated with specific venom immunotherapy (VIT), nevertheless, one died of HVA after VIT discontinuation, and the other during VIT; both patients had cardiovascular comorbidities and were taking beta-blockers and/or ACE inhibitors. Our results point to the importance of screening all high-risk individuals for underlying cMCD using highly sensitive molecular methods for peripheral blood KIT p.D816V variant detection, including severe HVA and possibly beekeepers, for proper management and the need for lifelong VIT to prevent unnecessary deaths. Patients at the highest risk of fatal HVA, with concomitant cardiovascular and cMCD comorbidities, might not be protected from field stings even during regular VIT. Therefore, two adrenaline autoinjectors and lifelong VIT, and possibly cotreatment with omalizumab, should be considered for high-risk patients to prevent fatal HVA episodes. fatal anaphylaxis Hymenoptera venom mastocytosis venom immunotherapy medicina smrtna anafilaksija strup Hymenoptera mastocitoza imunoterapija s strupi imunoterapija true false true Angleški jezik Slovenski jezik Članek v reviji 2024-11-13 13:29:30 2024-11-13 13:29:30 2024-11-22 14:56:20 0000-00-00 00:00:00 2023 0 0 9 str. iss. 22, art. 16368 Vol. 24 Nov. 2023 0000-00-00 Zaloznikova Objavljeno NiDoloceno 0000-00-00 0000-00-00 0000-00-00 616 1422-0067 10.3390/ijms242216368 174252803 ijms-24-16368.pdf ijms-24-16368.pdf 1 C55B0E2AE6F6F19D8969F784B18E23DD 76c82482252fe83836c2372d7833860341af9db76474210d05757657d2c830e4 ebb0690f-a1ba-11ef-b232-0050569b8976 https://repozitorij.uni-lj.si/Dokument.php?lang=slv&id=195602 https://www.mdpi.com/1422-0067/24/22/16368 1 91634199-a1ba-11ef-b232-0050569b8976 https://repozitorij.uni-lj.si/Dokument.php?lang=slv&id=195601 Biotehniška fakulteta Medicinska fakulteta Fakulteta za farmacijo 0 0 0