Fatal Hymenoptera venom–triggered anaphylaxis in patients with unrecognized clonal mast cell disorder : is mastocytosis to blame?

Rijavec, Matija (avtor), Inkret, Jezerka (avtor), Bidovec-Stojković, Urška (avtor), Carli, Tanja (avtor), Frelih, Nina (avtor), Kukec, Andreja (avtor), Korošec, Peter (avtor), Košnik, Mitja (avtor)

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.

• Jezik: Angleški jezik
• Ključne besede: fatal anaphylaxis, Hymenoptera venom, mastocytosis, venom immunotherapy
• Vrsta gradiva: Članek v reviji
• Tipologija: 1.01 - Izvirni znanstveni članek
• Organizacija: BF - Biotehniška fakulteta ; MF - Medicinska fakulteta ; FFA - Fakulteta za farmacijo
• Status publikacije: Objavljeno
• Različica publikacije: Objavljena publikacija
• Leto izida: 2023
• Št. strani: 9 str.
• Številčenje: Vol. 24, iss. 22, art. 16368
• PID: 20.500.12556/RUL-164839
• UDK: 616
• ISSN pri članku: 1422-0067
• DOI: 10.3390/ijms242216368
• COBISS.SI-ID: 174252803
• Datum objave v RUL: 13.11.2024

Gradivo je del revije

• Naslov: International journal of molecular sciences
• Skrajšan naslov: Int. j. mol. sci.
• Založnik: MDPI
• ISSN: 1422-0067
• COBISS.SI-ID: 2779162

Licence

• Licenca: CC BY 4.0, Creative Commons Priznanje avtorstva 4.0 Mednarodna
• Povezava: http://creativecommons.org/licenses/by/4.0/deed.sl
• Opis: To je standardna licenca Creative Commons, ki daje uporabnikom največ možnosti za nadaljnjo uporabo dela, pri čemer morajo navesti avtorja.

Sekundarni jezik

• Jezik: Slovenski jezik
• Ključne besede: medicina, smrtna anafilaksija, strup Hymenoptera, mastocitoza, imunoterapija s strupi, imunoterapija

Projekti

• Financer: ARIS - Javna agencija za znanstvenoraziskovalno in inovacijsko dejavnost Republike Slovenije
• Številka projekta: P3-0360
• Naslov: Celostna obravnava alergijskih bolezni in astme v Sloveniji: od epidemiologije do genetike