{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T22:52:21Z","timestamp":1792450341147,"version":"4.5.0"},"reference-count":58,"publisher":"American Society of Hematology","issue":"2","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,1,14]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mastocytosis is a hematopoietic neoplasm characterized by expansion of KIT D816V-mutated clonal mast cells in various organs and severe or even life-threatening anaphylactic reactions. Recently, hereditary \u03b1-tryptasemia (H\u03b1T) has been described as a common genetic trait with increased copy numbers of the \u03b1-tryptase encoding gene, TPSAB1, and associated with an increased basal serum tryptase level and a risk of mast cell activation. The purpose of our study was to elucidate the clinical relevance of H\u03b1T in patients with mastocytosis. TPSAB1 germline copy number variants were assessed by digital polymerase chain reaction in 180 mastocytosis patients, 180 sex-matched control subjects, 720 patients with other myeloid neoplasms, and 61 additional mastocytosis patients of an independent validation cohort. \u03b1-Tryptase encoding TPSAB1 copy number gains, compatible with H\u03b1T, were identified in 17.2% of mastocytosis patients and 4.4% of the control population (P &amp;lt; .001). Patients with H\u03b1T exhibited higher tryptase levels than patients without H\u03b1T (median tryptase in H\u03b1T+ cases: 49.6 ng\/mL vs H\u03b1T\u2212 cases: 34.5 ng\/mL, P = .004) independent of the mast cell burden. Hymenoptera venom hypersensitivity reactions and severe cardiovascular mediator-related symptoms\/anaphylaxis were by far more frequently observed in mastocytosis patients with H\u03b1T than in those without H\u03b1T. Results were confirmed in an independent validation cohort. The high prevalence of H\u03b1T in mastocytosis hints at a potential pathogenic role of germline \u03b1-tryptase encoding TPSAB1 copy number gains in disease evolution. Together, our data suggest that H\u03b1T is a novel emerging robust biomarker in mastocytosis that is useful for determining the individual patient\u00b4s risk of developing severe anaphylaxis.<\/jats:p>","DOI":"10.1182\/blood.2020006157","type":"journal-article","created":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T21:14:53Z","timestamp":1597094093000},"page":"238-247","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":186,"title":["Hereditary \u03b1 tryptasemia is a valid genetic biomarker for severe mediator-related symptoms in mastocytosis"],"prefix":"10.1182","volume":"137","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0917-4117","authenticated-orcid":false,"given":"Georg","family":"Greiner","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria;"},{"name":"Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bettina","family":"Sprinzl","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Hematology and Oncology at the Hanusch Hospital, Center for Medical Genetics, Hanusch Hospital, Vienna, Austria;"},{"name":"Center for Medical Genetics, Hanusch Hospital, Vienna, Austria;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleksandra","family":"G\u00f3rska","sequence":"additional","affiliation":[{"name":"Department of Allergology, Medical University of Gda\u0144sk, Gda\u0144sk, Poland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1910-1018","authenticated-orcid":false,"given":"Franz","family":"Ratzinger","sequence":"additional","affiliation":[{"name":"Ihr Labor, Medical Diagnostic Laboratories, Vienna, 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