{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T05:19:49Z","timestamp":1789017589073,"version":"build-2803163510"},"reference-count":19,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2017,3,10]],"date-time":"2017-03-10T00:00:00Z","timestamp":1489104000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2017,3,10]],"date-time":"2017-03-10T00:00:00Z","timestamp":1489104000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Blood Cancer Journal"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    In 2012, the International Working Group for Myeloproliferative Neoplasms (MPN) Research and Treatment (IWG-MRT) reported an associations between mild bone marrow (BM) fibrosis (\u2a7egrade 1) in polycythemia vera (PV) and a lower incidence of thrombosis during the clinical course and a higher risk of fibrotic progression. The objective in the current study of 262 patients with PV was to validate these observations and also identify other risk factors for myelofibrosis-free survival (MFFS). About 127 (48%) patients displayed \u2a7egrade 1 reticulin fibrosis at the time of diagnosis; presenting clinical and laboratory features were not significantly different between patients with or without BM fibrosis. In univariate analysis, BM fibrosis had no significant impact on overall, leukemia-free or thrombosis-free survival, whereas a significant association was noted for MFFS (\n                    <jats:italic>P<\/jats:italic>\n                    =0.009, hazard ratio 2.9; 95% confidence interval 1.32\u20136.78); other risk factors for MFFS included leukocytosis \u2a7e15 \u00d7 10\n                    <jats:sup>9<\/jats:sup>\n                    \/l, presence of palpable splenomegaly and abnormal karyotype. During multivariable analysis, leukocytosis \u2a7e15 \u00d7 10\n                    <jats:sup>9<\/jats:sup>\n                    \/l, palpable splenomegaly and \u2a7egrade 1 BM reticulin fibrosis remained significant. The current study validates the previously observed association between \u2a7egrade 1 BM reticulin fibrosis in PV and subsequent fibrotic progression, and identifies leukocytosis and palpable splenomegaly as additional risk factors for fibrotic progression; additional studies are required to clarify the impact of BM fibrosis on thrombosis and that of abnormal karyotype on MFFS.\n                  <\/jats:p>","DOI":"10.1038\/bcj.2017.17","type":"journal-article","created":{"date-parts":[[2017,3,10]],"date-time":"2017-03-10T09:21:59Z","timestamp":1489137719000},"page":"e538-e538","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations"],"prefix":"10.1038","volume":"7","author":[{"given":"D","family":"Barraco","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Cerquozzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C A","family":"Hanson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R P","family":"Ketterling","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Pardanani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N","family":"Gangat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Tefferi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2017,3,10]]},"reference":[{"key":"BFbcj201717_CR1","doi-asserted-by":"publisher","first-page":"94","DOI":"10.1002\/ajh.24607","volume":"92","author":"A Tefferi","year":"2017","unstructured":"Tefferi A, Barbui T . Polycythemia vera and essential thrombocythemia: 2017 update on diagnosis, risk-stratification, and management. Am J Hematol 2017; 92: 94\u2013108.","journal-title":"Am J Hematol"},{"key":"BFbcj201717_CR2","doi-asserted-by":"publisher","first-page":"1874","DOI":"10.1038\/leu.2013.163","volume":"27","author":"A Tefferi","year":"2013","unstructured":"Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia 2013; 27: 1874\u20131881.","journal-title":"Leukemia"},{"key":"BFbcj201717_CR3","doi-asserted-by":"publisher","first-page":"e366","DOI":"10.1038\/bcj.2015.95","volume":"5","author":"S Cerquozzi","year":"2015","unstructured":"Cerquozzi S, Tefferi A . Blast transformation and fibrotic progression in polycythemia vera and essential thrombocythemia: a literature review of incidence and risk factors. 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