{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T02:41:51Z","timestamp":1788316911397,"version":"build-2803163510"},"reference-count":19,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2025,7,3]],"date-time":"2025-07-03T00:00:00Z","timestamp":1751500800000},"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":[[2025,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>The current study includes 910 patients with systemic mastocytosis (SM) seen at the Mayo Clinic from 1968 to 2024. The primary objective was to examine the prognostic contribution of the International Consensus Classification (ICC), in the context of the Mayo Alliance Prognostic System (MAPS) for SM. World Health Organization classification (WHO\u2010HAEM5) subcategories included (i) indolent\/smoldering SM (ISM\/SSM; <jats:italic>N<\/jats:italic>\u2009=\u2009518), (ii) SM associated with another hematological neoplasm (SM\u2010AHN; <jats:italic>N<\/jats:italic>\u2009=\u2009273), with the latter including both myeloid and lymphoid neoplasms, (iii) aggressive SM (ASM; <jats:italic>N<\/jats:italic>\u2009=\u2009106), and (iv) WHO\u2010defined mast cell leukemia (MCL; <jats:italic>N<\/jats:italic>\u2009=\u200913), which included mast cells with both \u201cmature\u201d and \u201cimmature\u201d morphology. The ICC\u2010defined subcategories were mostly similar with the exception that SM\u2010AHN was replaced by SM associated with another myeloid neoplasm (SM\u2010AMN; <jats:italic>N<\/jats:italic>\u2009=\u2009235) and WHO\u2010defined MCL was replaced by ICC\u2010defined MCL (<jats:italic>N<\/jats:italic>\u2009=\u20098), which included only those with immature MC morphology. Overall survival (OS) was similar between WHO\u2010defined MCL (median 1.8\u2009years) vs. SM\u2010AHN (median 2.3\u2009years; <jats:italic>p<\/jats:italic>\u2009=\u20090.3) but significantly different between ICC\u2010defined MCL (median 0.08\u2009years) vs. SM\u2010AMN (median 2.0\u2009years; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01). Significant difference in OS was also apparent between ICC\u2010defined SM\u2010AMN and SM associated with lymphoid neoplasm (SM\u2010ALN; median 8.1\u2009years; HR 3.4; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01). Multivariable analysis confirmed the inter\u2010independent prognostic contributions of both ICC\u2010defined morphologic subcategories and MAPS risk variables, including age &gt;\u200960\u2009years, anemia, alkaline phosphatase\u2009&gt;\u2009ULN, and platelet count &lt;\u2009150\u2009\u00d7\u200910<jats:sup>9<\/jats:sup>\/L (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.03 in all instances); the same was not true for WHO\u2010HAEM5\u2010defined SM subcategories. The addition of mutational information into the multivariable model resulted in ousting anemia and inclusion of <jats:italic>ASXL1<\/jats:italic> (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01), <jats:italic>SRSF2<\/jats:italic> (<jats:italic>p<\/jats:italic>\u2009=\u20090.01), and <jats:italic>NRAS<\/jats:italic> (<jats:italic>p<\/jats:italic>\u2009=\u20090.01) mutations as additional risk factors. Classification of SM by ICC is prognostically more accurate, compared to WHO\u2010HAEM5, and strengthens the prognostic contribution of morphology to current clinical and molecular risk models.<\/jats:p>","DOI":"10.1002\/ajh.27764","type":"journal-article","created":{"date-parts":[[2025,7,3]],"date-time":"2025-07-03T08:14:51Z","timestamp":1751530491000},"page":"1566-1576","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Systemic Mastocytosis in 910 Patients: Prognostic Contribution of the International Consensus Classification in the Context of the Mayo Alliance Prognostic System"],"prefix":"10.1002","volume":"100","author":[{"given":"Fnu","family":"Aperna","sequence":"first","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maymona G.","family":"Abdelmagid","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahesh","family":"Kumar","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph H.","family":"Butterfield","sequence":"additional","affiliation":[{"name":"Allergy and Immunology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thanai","family":"Pongdee","sequence":"additional","affiliation":[{"name":"Allergy and Immunology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7441-5140","authenticated-orcid":false,"given":"Cecilia Y.","family":"Arana Yi","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology Mayo Clinic  Phoenix Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6998-662X","authenticated-orcid":false,"given":"Mrinal S.","family":"Patnaik","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2160-9689","authenticated-orcid":false,"given":"Daniel A.","family":"Arber","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Chicago  Chicago Illinois USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"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":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7627-9560","authenticated-orcid":false,"given":"Dong","family":"Chen","sequence":"additional","affiliation":[{"name":"Hematopathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaaren K.","family":"Reichard","sequence":"additional","affiliation":[{"name":"Hematopathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9104-6172","authenticated-orcid":false,"given":"Naseema","family":"Gangat","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9084-4148","authenticated-orcid":false,"given":"Animesh","family":"Pardanani","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4605-3821","authenticated-orcid":false,"given":"Ayalew","family":"Tefferi","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,7,3]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41577-022-00731-2"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26962"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/0009922808318344"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-022-01014-w"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022015850"},{"key":"e_1_2_10_7_1","volume-title":"WHO Classification of Tumours","author":"Haematolymphoid Tumors","year":"2024"},{"key":"e_1_2_10_8_1","volume-title":"The International Consensus Classification of Myeloid and Lymphoid Neoplasms","author":"Arber D. 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