{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,17]],"date-time":"2025-10-17T14:27:21Z","timestamp":1760711241787,"version":"3.40.5"},"reference-count":21,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T00:00:00Z","timestamp":1695945600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The World Health Organization (WHO) classification system categorizes advanced systemic mastocytosis (SM\u2010Adv) into aggressive SM (ASM), mast cell leukemia (MCL), and SM with associated hematological neoplasm (SM\u2010AHN). By contrast, the International Consensus Classification (ICC) requires \u201cimmature\u201d MC cytomorphology for the diagnosis of MCL and limits SM\u2010AHN to myeloid neoplasms (SM\u2010AMN). The current study includes 329 patients with SM\u2010Adv (median age 65\u2009years, range 18\u201388; males 58%): WHO subcategories SM\u2010AHN (<jats:italic>N<\/jats:italic>\u2009=\u2009212; 64%), ASM (<jats:italic>N<\/jats:italic>\u2009=\u200999; 30%), and MCL (<jats:italic>N<\/jats:italic>\u2009=\u200918; 6%); ICC subcategories SM\u2010AMN (<jats:italic>N<\/jats:italic>\u2009=\u2009190; 64%), ASM (N\u2009=\u200999; 33%), and MCL (<jats:italic>N<\/jats:italic>\u2009=\u20099; 3%); WHO\u2010defined MCL with \u201cmature\u201d MC cytomorphology and SM\u2010AHN associated with lymphoid neoplasms were operationally labeled as \u201cMCL\u2010mature\u201d (<jats:italic>N<\/jats:italic>\u2009=\u20099) and SM\u2010ALN (<jats:italic>N<\/jats:italic>\u2009=\u200922), respectively, and distinguished from ICC\u2010defined MCL and SM\u2010AMN. Multivariable analysis that included the Mayo alliance risk factors for survival in SM (age &gt;60\u2009years, anemia, thrombocytopenia, increased alkaline phosphatase) revealed more accurate survival prediction with the ICC versus WHO classification order: (i) survival was significantly worse with MCL\u2010immature versus MCL\u2010mature (hazard ratio [HR] 15; <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.01), (ii) prognostic distinction between MCL and SM\u2010AHN\/AMN was confirmed in the context of ICC (HR 9.3; <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.01) but not WHO classification order (<jats:italic>p<\/jats:italic>\u2009=\u2009.99), (iii) survival was similar between MCL\u2010mature and SM\u2010AMN (<jats:italic>p<\/jats:italic>\u2009=\u2009.18), and (iv) SM\u2010AMN (HR 1.7; <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.01) but not SM\u2010ALN (<jats:italic>p<\/jats:italic>\u2009=\u2009.37) was prognostically distinct from ASM. The current study provides evidence for the independent prognostic contribution of both the ICC system for SM\u2010Adv and the Mayo alliance risk factors for survival in SM.<\/jats:p>","DOI":"10.1002\/ajh.27113","type":"journal-article","created":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T08:49:24Z","timestamp":1695977364000},"page":"21-27","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Granularity in disease classification impacts survival prediction in advanced systemic mastocytosis: A single institution study of 329 informative cases"],"prefix":"10.1002","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4605-3821","authenticated-orcid":false,"given":"Ayalew","family":"Tefferi","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Medicine Mayo Clinic  Rochester Minnesota 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Hematology, Department of Medicine Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2730-8593","authenticated-orcid":false,"given":"Kebede","family":"Begna","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9104-6172","authenticated-orcid":false,"given":"Naseema","family":"Gangat","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1660-7013","authenticated-orcid":false,"given":"Attilio","family":"Orazi","sequence":"additional","affiliation":[{"name":"Texas Tech University Health Sciences Center  El Paso Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7627-9560","authenticated-orcid":false,"given":"Dong","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Hematopathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaaren K.","family":"Reichard","sequence":"additional","affiliation":[{"name":"Division of Hematopathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9084-4148","authenticated-orcid":false,"given":"Animesh","family":"Pardanani","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine Mayo Clinic  Rochester Minnesota 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