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In most cases, neoplastic cells exhibit the transforming\n                      <jats:italic>KIT<\/jats:italic>\n                      mutation D816V. Clinical symptoms arise from organ infiltration by neoplastic MC and\/or pro-inflammatory mediators and cytokines released by these cells. Indeed, in a majority of the patients, acute or chronic mediator-induced symptoms are recorded, and in some instances, symptoms are severe and drug-resistant or even manifest as life-threatening anaphylaxis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Purpose of review<\/jats:title>\n                    <jats:p>In this article, we review the role of MC and basophils in anaphylactic reactions in patients with SM and the impact of genetic variables, co-morbidities, specific IgE, and factors counteracting MC activation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Recent findings and summary<\/jats:title>\n                    <jats:p>Whereas avoidance of all known and potential triggers of MC activation is crucial in the management of anaphylaxis in SM patients, specific therapy is also standard, including histamine receptor blockers, other anti-mediator-type drugs and drugs suppressing MC activation and\/or expansion. Novel KIT D816V-targeting drugs can potentially decrease the anaphylaxis risk by reducing the MC burden and by targeting KIT-dependent and IgE-receptor-dependent signaling processes in neoplastic MC. Application of such drugs often leads to sustained responses and an increase in the quality of life in these patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s11882-026-01257-6","type":"journal-article","created":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T06:53:00Z","timestamp":1771311180000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Systemic Mastocytosis, KIT and the Effects of KIT Tyrosine Kinase Inhibitors on Mast Cell and Basophil Activation"],"prefix":"10.1007","volume":"26","author":[{"given":"Peter","family":"Valent","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karin","family":"Hartmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriele","family":"Stefanzl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y\u00fcksel","family":"Filik","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karin","family":"Bauer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lina","family":"Degenfeld-Schonburg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karoline V.","family":"Gleixner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Siebenhaar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marek","family":"Niedoszytko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang R.","family":"Sperr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Narges","family":"Aghaallaei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregor","family":"Hoermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vito","family":"Sabato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Orfao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Arock","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cem","family":"Akin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,2,17]]},"reference":[{"key":"1257_CR1","first-page":"5","volume":"62","author":"SJ Galli","year":"1990","unstructured":"Galli SJ. 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K.H. was supported by the Swiss National Science Foundation (SNSF) grant 310030_207705. V.S. is Senior Investigator of the Flemish Research Council (FWO, grant 1804523N).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Grant Support"}},{"value":"P.V. received research grant from AOP Orphan, and advisory board and other consultancy honoraria from Novartis, Blueprint Medicines, Cogent, Pfizer, AOP Orphan, Stemline, Abbvie, Delbert, Pentabase, Daiichi-Sankyo, Amgen. C.A. received research funding from Blueprint Medicines, Cogent and Telios, and has consultancy agreements with Blueprint Medicines, Cogent, Telios, and Novartis. Karin Hartmann: Advisory board and consultant honoraria: ALK-Abello, Allergopharma, Almirall, BioCryst, Blueprint, Cogent, Galderma, KalVista, Leo, Menarini, Novartis, Pfizer, Sanofi, Takeda, and Thermo Fisher; Gabriele Stefanzl: no COI; Y\u00fcksel Filik: no COI; Karin Bauer: no COI; Lina-Degenfeld-Schonburg: no COI; Karoline V. Gleixner: Advisory board honoraria: Jazz Pharmaceuticals, AbbVie, Novartis, Servier, Blueprint, Incyte, Celgene, Astellas, Daiichi Sankyo, BMS; Frank Siebenhaar: Advisory board, consultancy and other honoraria or research funding: Allakos, Alys, Blueprint, Celldex, Cogent, Escient, Granular, GSK, Invea, Medison, Moxie, Noucor, Novartis, Pentabase, Pharvaris, Regeneron, Sanofi, Swixx, Telios, Third Harmonic Bio, UCB; Marek Niedoszytko: Consultancy in clinical trials: Novartis, AB Science, Telios, Blueprint, ABM; Wolfgang R. Sperr: 1. Consultancy and Honoraria: Thermo Fisher, AbbVie, Novartis, Pfizer, BMS\/Celgene, Blueprint, Incyte, Stemline, Servier, Jazz, Teva, 2. Research Grant: Pfizer; Narges Aghaallaei: no COI; Gregor Hoermann: Advisory Board and Honoraria: AOP Orphan Pharmaceuticals, AstraZeneca, Blueprint Medicines, Cogent Biosciences, Gilead, Janssen-Cilag Pharma, Jazz Pharmaceuticals, MSD Sharp & Dohme; Vito Sabato: 1. Is a speaker\/advisor for Blueprint Medicines Corporation and Cogent and and received honoraria from Blueprint Medicines Corporation, Cogent, and Novartis 2. Research funding from Blueprint Medicines Corporation. Alberto Orfao: 1. Research grants: Blueprint Medicines, ImmunosStep, 2. Advisory Board and Honoraria: Blueprint Medicines; Michel Arock: 1. Research Grant: Blueprint Medicines, 2. Advisory Board and Honoraria: AB Science, Blueprint Medicines, Novartis, Thermo Fisher. Cem Akin (CA): received consulting fees and research support from Blueprint Medicines, Sanofi company, Cogent Biosciences, and Telios, and consulting fees from Novartis. CA is also a Section Editor of \u201cCurrent Allergy and Asthma Reports\u201d. He was not involved in the peer-review or handling of the manuscript. The authors have no other competing interests to disclose.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}],"article-number":"10"}}