{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T14:48:24Z","timestamp":1785422904853,"version":"3.56.0"},"reference-count":116,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2023,10,25]],"date-time":"2023-10-25T00:00:00Z","timestamp":1698192000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Konsul T.H.C. Bergh Foundation, Sweden"},{"name":"Swedish Society of Medicine, Sweden"},{"name":"regional agreement on medical training and clinical research (ALF) between Stockholm County Council and Karolinska Institutet, Stockholm, Sweden"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Our knowledge of biology and mast cell (MC) function, as well as disorders associated with the pathologic activation of MCs, has evolved over the last few decades. Anaphylaxis, mast cell activation syndrome (MCAS), and mastocytosis are interrelated yet distinct conditions within the spectrum of mast cell activation disorders. Nevertheless, all three conditions can co-exist in one and the same patient, as pathologic MC activation is the key finding in all three. When mediator release is excessive and involves multiple systems, anaphylaxis and MCAS may occur. Furthermore, mastocytosis is a clonal disorder of MCs and often presents with anaphylaxis and MCAS. Nevertheless, in some cases, even the proliferative and accumulative features of MCs in mastocytosis can account for symptoms and disease progression. In each case, diagnosis can be only made when the diagnostic consensus criteria are fulfilled. The current article aims to provide a concise clinical update and pinpoint the main difficulties in diagnosing these puzzling disorders of MCs in medical practice.<\/jats:p>","DOI":"10.3390\/diagnostics13213307","type":"journal-article","created":{"date-parts":[[2023,10,25]],"date-time":"2023-10-25T09:54:08Z","timestamp":1698227648000},"page":"3307","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["A Puzzling Mast Cell Trilogy: Anaphylaxis, MCAS, and Mastocytosis"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1683-8882","authenticated-orcid":false,"given":"Theo","family":"G\u00fclen","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine and Allergy, Karolinska University Hospital Huddinge, 141 86 Stockholm, Sweden"},{"name":"Department of Medicine Solna, Division of Immunology and Allergy, Karolinska Institutet, 171 77 Stockholm, Sweden"},{"name":"Department of Medicine Huddinge, Karolinska Institutet, 141 52 Stockholm, Sweden"},{"name":"Mastocytosis Centre Karolinska, Karolinska University Hospital Huddinge, 141 86 Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"10743","DOI":"10.7150\/thno.46719","article-title":"Mast cells as a unique hematopoietic lineage and cell system: From Paul Ehrlich\u2019s visions to precision medicine concepts","volume":"10","author":"Valent","year":"2020","journal-title":"Theranostics"},{"key":"ref_2","first-page":"13","article-title":"The mast cell: A multifunctional effector cell","volume":"7","author":"Crivellato","year":"2003","journal-title":"Adv. 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