{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T23:16:34Z","timestamp":1775690194509,"version":"3.50.1"},"reference-count":45,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2024,4,10]],"date-time":"2024-04-10T00:00:00Z","timestamp":1712707200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,9,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>Myeloproliferative neoplasm, unclassifiable (MPN-U, revised to MPN, not otherwise specified in the fifth edition of the World Health Organization classification) is a heterogeneous category of primary marrow disorders with clinical, morphologic, and\/or molecular features that preclude classification as a more specific MPN subtype due to stage at diagnosis, overlapping features between MPN subtypes, or the presence of coexisting disorders. Compared with other MPN subtypes, the contribution of the mutational landscape in MPN-U in conjunction with other clinical and morphologic biomarkers to prognosis has been less well investigated.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We performed a multicenter, retrospective study of MPN-U (94 cases) to better define the clinicopathologic features, genetic landscape, and clinical outcomes, including subgroups of early-stage, advanced-stage, and coexisting disorders. The Dynamic International Prognostic Scoring System (DIPSS) plus scoring system was applied to assess its relevance to MPN-U prognosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Multivariate analysis demonstrated bone marrow blast count and DIPSS plus score as statistically significant in predicting overall survival. Univariate analysis identified additional potential poor prognostic markers, including abnormal karyotype and absence of JAK2 mutation. Secondary mutations were frequent in the subset analyzed by next-generation sequencing (26\/37 cases, 70.3%) with a borderline association between high molecular risk mutations and overall survival.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>This study, as one of the largest of MPN-U studies incorporating both clinicopathologic and molecular data, moves toward identification of biomarkers that better predict prognosis in this heterogeneous category.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ajcp\/aqae033","type":"journal-article","created":{"date-parts":[[2024,4,10]],"date-time":"2024-04-10T12:34:40Z","timestamp":1712752480000},"page":"233-242","source":"Crossref","is-referenced-by-count":7,"title":["Predictors of clinical outcome in myeloproliferative neoplasm, unclassifiable: A Bone Marrow Pathology Group study"],"prefix":"10.1093","volume":"162","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9274-0214","authenticated-orcid":false,"given":"Genevieve M","family":"Crane","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine, Cleveland Clinic , Cleveland, OH , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4424-6139","authenticated-orcid":false,"given":"Julia T","family":"Geyer","sequence":"additional","affiliation":[{"name":"Department of Pathology, Weill Cornell Medicine , New York, NY , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beenu","family":"Thakral","sequence":"additional","affiliation":[{"name":"Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sa A","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Geoffrey D","family":"Wool","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Chicago , Chicago, IL , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ke David","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Utah , Salt Lake City, UT , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adam R","family":"Davis","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of Pennsylvania , Philadelphia, PA , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leonardo","family":"Boiocchi","sequence":"additional","affiliation":[{"name":"Department of Pathology, Massachusetts General Hospital , Boston, MA , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Bosler","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Cleveland Clinic , Cleveland, OH , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlos E","family":"Bueso-Ramos","sequence":"additional","affiliation":[{"name":"Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel A","family":"Arber","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Chicago , Chicago, IL , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tracy I","family":"George","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Utah , Salt Lake City, UT , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adam","family":"Bagg","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of Pennsylvania , Philadelphia, PA , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert P","family":"Hasserjian","sequence":"additional","affiliation":[{"name":"Department of Pathology, Massachusetts General Hospital , Boston, MA , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Attilio","family":"Orazi","sequence":"additional","affiliation":[{"name":"Department of Pathology, Texas Tech University Health Science Center , El Paso, TX , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8623-4067","authenticated-orcid":false,"given":"Eric D","family":"Hsi","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, MN , US"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heesun J","family":"Rogers","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Cleveland Clinic , Cleveland, OH , US"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,4,10]]},"reference":[{"key":"2024090305544893100_CIT0001","first-page":"166","article-title":"The International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: myeloproliferative neoplasms","volume-title":"Am J Hematol.","author":"Thiele","year":"2023"},{"key":"2024090305544893100_CIT0002","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues.","author":"Swerdlow","year":"2017","edition":"4"},{"key":"2024090305544893100_CIT0003","first-page":"E48","article-title":"Impact of the 2016 revised WHO criteria for myeloproliferative neoplasms, unclassifiable: comparison with the 2008 version","volume-title":"Am J 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polycythemia vera, and myeloproliferative neoplasm, unclassifiable","volume-title":"Am J Clin Pathol.","author":"Kim","year":"2015"},{"key":"2024090305544893100_CIT0011","first-page":"680","article-title":"Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms","volume-title":"Blood.","author":"Rumi","year":"2017"},{"key":"2024090305544893100_CIT0012","first-page":"2228","article-title":"Genomic profiling for clinical decision making in myeloid neoplasms and acute leukemia","volume-title":"Blood.","author":"Duncavage","year":"2022"},{"key":"2024090305544893100_CIT0013","first-page":"110","article-title":"JAK2 (and other genes) be nimble with MPN diagnosis, prognosis, and therapy","volume-title":"Hematology Am Soc Hematol Educ Program.","author":"Ciboddo","year":"2018"},{"key":"2024090305544893100_CIT0014","first-page":"1142","article-title":"Impact of high-molecular-risk mutations on transplantation outcomes in patients with myelofibrosis","volume-title":"Biol Blood Marrow Transplant.","author":"Tamari","year":"2019"},{"key":"2024090305544893100_CIT0015","first-page":"1804","article-title":"The number of prognostically detrimental mutations and prognosis in primary myelofibrosis: an international study of 797 patients","volume-title":"Leukemia.","author":"Guglielmelli","year":"2014"},{"key":"2024090305544893100_CIT0016","first-page":"5227","article-title":"EZH2 mutational status predicts poor survival in myelofibrosis","volume-title":"Blood.","author":"Guglielmelli","year":"2011"},{"key":"2024090305544893100_CIT0017","first-page":"444","article-title":"Comparison of the mutational profiles of primary myelofibrosis, polycythemia vera, and essential thrombocytosis","volume-title":"Am J Clin Pathol.","author":"Song","year":"2017"},{"key":"2024090305544893100_CIT0018","first-page":"1861","article-title":"Mutations and prognosis in primary myelofibrosis","volume-title":"Leukemia.","author":"Vannucchi","year":"2013"},{"key":"2024090305544893100_CIT0019","first-page":"4887","article-title":"Leukemic evolution of polycythemia vera and essential thrombocythemia: genomic profiles predict time to transformation","volume-title":"Blood Adv.","author":"Luque Paz","year":"2020"},{"key":"2024090305544893100_CIT0020","first-page":"2927","article-title":"ASXL1 mutations are prognostically significant in PMF, but not MF following essential thrombocythemia or polycythemia vera","volume-title":"Blood Adv.","author":"Guglielmelli","year":"2022"},{"key":"2024090305544893100_CIT0021","first-page":"1442","article-title":"Genomic analysis of primary and secondary myelofibrosis redefines the prognostic impact of ASXL1 mutations: a FIM study","volume-title":"Blood Adv.","author":"Luque Paz","year":"2021"},{"key":"2024090305544893100_CIT0022","first-page":"1416","article-title":"Classification and personalized prognosis in myeloproliferative neoplasms","volume-title":"N Engl J Med.","author":"Grinfeld","year":"2018"},{"issue":"100016","key":"2024090305544893100_CIT0023","first-page":"1","article-title":"Triple-negative primary myelofibrosis: a bone marrow pathology group study","volume":"36","author":"Al-Ghamdi","year":"2023","journal-title":"Mod Pathol."},{"key":"2024090305544893100_CIT0024","first-page":"1851","article-title":"Molecular landscape and clonal architecture of adult myelodysplastic\/myeloproliferative neoplasms","volume-title":"Blood.","author":"Palomo","year":"2020"},{"key":"2024090305544893100_CIT0025","first-page":"2100","article-title":"Mutational landscape of myelodysplastic\/myeloproliferative neoplasm-unclassifiable","volume-title":"Blood.","author":"Bose","year":"2018"},{"key":"2024090305544893100_CIT0026","first-page":"2895","article-title":"New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and 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myelofibrosis","volume-title":"Leukemia.","author":"Tefferi","year":"2018"},{"key":"2024090305544893100_CIT0034","volume-title":"An International System for Human Cytogenomic Nomenclature"},{"key":"2024090305544893100_CIT0035","first-page":"100016","article-title":"Triple-negative primary myelofibrosis: a bone marrow pathology group study","volume-title":"Mod Pathol.","author":"Al-Ghamdi","year":"2023"},{"key":"2024090305544893100_CIT0036","first-page":"552","article-title":"Mutations in MPNs: prognostic implications, window to biology, and impact on treatment decisions","volume-title":"Hematology Am Soc Hematol Educ Program.","author":"Shammo","year":"2016"},{"key":"2024090305544893100_CIT0037","first-page":"1062","article-title":"Clinical effect of driver mutations of JAK2, CALR, or MPL in primary myelofibrosis","volume-title":"Blood.","author":"Rumi","year":"2014"},{"key":"2024090305544893100_CIT0038","first-page":"264","article-title":"Improved survival of calreticulin-mutated patients compared with Janus kinase 2 in primary myelofibrosis: a meta-analysis","volume-title":"Clin Lymphoma Myeloma Leuk.","author":"Kourie","year":"2016"},{"key":"2024090305544893100_CIT0039","first-page":"1472","article-title":"CALR vs JAK2 vs MPL-mutated or triple-negative myelofibrosis: clinical, cytogenetic and molecular comparisons","volume-title":"Leukemia.","author":"Tefferi","year":"2014"},{"key":"2024090305544893100_CIT0040","first-page":"348","article-title":"Driver mutations and prognosis in primary myelofibrosis: Mayo-Careggi MPN alliance study of 1,095 patients","volume-title":"Am J Hematol.","author":"Tefferi","year":"2018"},{"key":"2024090305544893100_CIT0041","first-page":"156","article-title":"Splanchnic vein thromboses associated with myeloproliferative neoplasms: an international, retrospective study on 518 cases","volume-title":"Am J 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