{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T20:08:48Z","timestamp":1791576528315,"version":"4.3.4"},"reference-count":12,"publisher":"American Society of Hematology","issue":"21","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2018,11,13]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Systemic mastocytosis (SM) is a clinically heterogeneous disease with prognosis chiefly assigned based on World Health Organization (WHO) morphologic subclassification. We assessed the feasibility of developing contemporary risk models for SM based on clinical and integrated clinical-genetics information. Diagnosis of SM was per WHO criteria, and karyotype and next-generation sequencing data were available in a subset of the total 580 patients (median age, 55 years; range, 18-88 years) seen at the Mayo Clinic between 1968 and 2015. Morphologic subcategories were indolent\/smoldering in 291 (50%) and \u201cadvanced\u201d in 289 (50%): SM with an associated hematological neoplasm in 199, aggressive SM in 85, and mast cell leukemia in 5. Multivariable analysis of clinical variables identified age &amp;gt;60 years, advanced SM, thrombocytopenia &amp;lt;150 \u00d7 109\/L, anemia below sex-adjusted normal, and increased alkaline phosphatase (ALP) as independent risk factors for survival; respective hazard ratios (HRs) 95% confidence intervals (95% CIs) were 2.5 (1.9-3.4), 2.7 (1.8-4.0), 2.5 (1.9-3.4), 2.2 (1.6-3.1), and 2.1 (1.5-3.0). In addition, ASXL1 (HR, 4.5; 95% CI, 2.6-7.6), RUNX1 (HR, 4.3; 95% CI, 1.3-10.8), and NRAS (HR, 5.0, 95% CI, 1.5-13.2) mutations were independently associated with inferior survival. Combined clinical, cytogenetic, and molecular risk factor analysis confirmed the independent prognostic contribution of adverse mutations (2.6, 1.6-4.4), advanced SM (4.0, 1.8-10.0), thrombocytopenia (2.8, 1.7-4.5), increased ALP (2.1, 1.2-4.0), and age &amp;gt;60 years (2.2, 1.3-3.6). These data were subsequently used to develop clinical and hybrid clinical-molecular risk models. The current study advances 2 complementary risk models for SM and highlights the independent prognostic contribution of mutations.<\/jats:p>","DOI":"10.1182\/bloodadvances.2018026245","type":"journal-article","created":{"date-parts":[[2018,11,9]],"date-time":"2018-11-09T17:46:39Z","timestamp":1541785599000},"page":"2964-2972","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":80,"title":["Mayo alliance prognostic system for mastocytosis: clinical and hybrid clinical-molecular models"],"prefix":"10.1182","volume":"2","author":[{"given":"Animesh","family":"Pardanani","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sahrish","family":"Shah","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Mannelli","sequence":"additional","affiliation":[{"name":"Center of Research and Innovation of Myeloproliferative Neoplasms, University of Florence, Azienda Ospedaliero Universitaria Careggi, Florence, Italy; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoseph C.","family":"Elala","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Guglielmelli","sequence":"additional","affiliation":[{"name":"Center of Research and Innovation of Myeloproliferative Neoplasms, University of Florence, Azienda Ospedaliero Universitaria Careggi, Florence, Italy; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terra L.","family":"Lasho","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mrinal M.","family":"Patnaik","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naseema","family":"Gangat","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhett P.","family":"Ketterling","sequence":"additional","affiliation":[{"name":"Division of Laboratory Genetics and Genomics, Department of Laboratory Medicine, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaaren K.","family":"Reichard","sequence":"additional","affiliation":[{"name":"Division of Hematopathology, Department of Laboratory Medicine, Mayo Clinic, Rochester, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Curtis A.","family":"Hanson","sequence":"additional","affiliation":[{"name":"Division of Hematopathology, Department of Laboratory Medicine, Mayo Clinic, Rochester, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro M.","family":"Vannucchi","sequence":"additional","affiliation":[{"name":"Center of Research and Innovation of Myeloproliferative Neoplasms, University of Florence, Azienda Ospedaliero Universitaria Careggi, Florence, Italy; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayalew","family":"Tefferi","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","published-online":{"date-parts":[[2018,11,9]]},"reference":[{"issue":"20","key":"2020021108400635100_B1","doi-asserted-by":"crossref","first-page":"2391","DOI":"10.1182\/blood-2016-03-643544","article-title":"The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia","volume":"127","author":"Arber","year":"2016","journal-title":"Blood"},{"issue":"23","key":"2020021108400635100_B2","doi-asserted-by":"crossref","first-page":"5727","DOI":"10.1182\/blood-2009-02-205237","article-title":"Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors","volume":"113","author":"Lim","year":"2009","journal-title":"Blood"},{"issue":"9","key":"2020021108400635100_B3","doi-asserted-by":"crossref","first-page":"888","DOI":"10.1002\/ajh.24426","article-title":"Next-generation sequencing in systemic mastocytosis: derivation of a mutation-augmented clinical prognostic model for survival","volume":"91","author":"Pardanani","year":"2016","journal-title":"Am J Hematol"},{"issue":"3","key":"2020021108400635100_B4","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1111\/bjh.13865","article-title":"ASXL1 and CBL mutations are independently predictive of inferior survival in advanced systemic mastocytosis","volume":"175","author":"Pardanani","year":"2016","journal-title":"Br J Haematol"},{"key":"2020021108400635100_B5","article-title":"Cytogenetic abnormalities in systemic mastocytosis: WHO subcategory-specific incidence and prognostic impact among 348 informative cases [published online ahead of print 28 August 2018]","author":"Shah","journal-title":"Am J Hematol"},{"issue":"5","key":"2020021108400635100_B6","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1002\/gcc.22526","article-title":"Incidence and prognostic impact of cytogenetic aberrations in patients with systemic mastocytosis","volume":"57","author":"Naumann","year":"2018","journal-title":"Genes Chromosomes Cancer"},{"issue":"1","key":"2020021108400635100_B7","doi-asserted-by":"crossref","first-page":"e85362","DOI":"10.1371\/journal.pone.0085362","article-title":"ASXL1 but not TET2 mutations adversely impact overall survival of patients suffering systemic mastocytosis with associated clonal hematologic non-mast-cell diseases","volume":"9","author":"Damaj","year":"2014","journal-title":"PLoS One"},{"issue":"1","key":"2020021108400635100_B8","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1038\/leu.2015.284","article-title":"Additional mutations in SRSF2, ASXL1 and\/or RUNX1 identify a high-risk group of patients with KIT D816V(+) advanced systemic mastocytosis","volume":"30","author":"Jawhar","year":"2016","journal-title":"Leukemia"},{"issue":"8","key":"2020021108400635100_B9","doi-asserted-by":"crossref","first-page":"1138","DOI":"10.1038\/modpathol.2015.72","article-title":"Detection of the KIT D816V mutation in peripheral blood of systemic mastocytosis: diagnostic implications","volume":"28","author":"Jara-Acevedo","year":"2015","journal-title":"Mod Pathol"},{"issue":"26","key":"2020021108400635100_B10","doi-asserted-by":"crossref","first-page":"2530","DOI":"10.1056\/NEJMoa1513098","article-title":"Efficacy and safety of midostaurin in advanced systemic mastocytosis","volume":"374","author":"Gotlib","year":"2016","journal-title":"N Engl J Med"},{"key":"2020021108400635100_B11","first-page":"2","article-title":"Clinical activity in a phase 1 study of Blu-285, a potent, highly-selective inhibitor of KIT D816V in advanced systemic mastocytosis (AdvSM)","volume":"130","author":"DeAngelo","year":"2017","journal-title":"Blood"},{"issue":"2","key":"2020021108400635100_B12","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1182\/blood-2017-01-764423","article-title":"Response and progression on midostaurin in advanced systemic mastocytosis: KIT D816V and other molecular markers","volume":"130","author":"Jawhar","year":"2017","journal-title":"Blood"}],"container-title":["Blood Advances"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/ashpublications.org\/bloodadvances\/article-pdf\/2\/21\/2964\/1629912\/advances026245.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/ashpublications.org\/bloodadvances\/article-pdf\/2\/21\/2964\/1629912\/advances026245.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,2,11]],"date-time":"2020-02-11T14:15:30Z","timestamp":1581430530000},"score":1,"resource":{"primary":{"URL":"https:\/\/ashpublications.org\/bloodadvances\/article\/2\/21\/2964\/16221\/Mayo-alliance-prognostic-system-for-mastocytosis"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11,9]]},"references-count":12,"journal-issue":{"issue":"21","published-print":{"date-parts":[[2018,11,13]]}},"URL":"https:\/\/doi.org\/10.1182\/bloodadvances.2018026245","relation":{},"ISSN":["2473-9529","2473-9537"],"issn-type":[{"value":"2473-9529","type":"print"},{"value":"2473-9537","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,11,9]]}}}