{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T23:17:51Z","timestamp":1793402271521,"version":"4.5.0"},"reference-count":56,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2020,2,25]],"date-time":"2020-02-25T00:00:00Z","timestamp":1582588800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,2,25]],"date-time":"2020-02-25T00:00:00Z","timestamp":1582588800000},"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 J."],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In the 2016 revised classification of myeloproliferative neoplasms pre-fibrotic primary myelofibrosis (pre-PMF) was recognized as a separate entity, distinct from essential thrombocythemia (ET). Owing that the majority of cases falling in the pre-PMF category were previously diagnosed as ET, one may question about the need to re-evaluate the results of epidemiologic, clinical, and molecular studies, and the results of clinical trials in the two entities. Based on a critical review of recently published studies, pre-PMF usually presents with a distinct clinical and hematological presentation and higher frequency of constitutional symptoms. <jats:italic>JAK2V617F<\/jats:italic> and <jats:italic>CALR<\/jats:italic> mutations in pre-PMF patients are superimposable to ET, whereas non-driver high-risk mutations are enriched in pre-PMF compared with ET. Thrombosis is not significantly different, whereas bleeding is more frequent in pre-PMF. Median survival is significantly shorter in pre-PMF and 10-year cumulative rates progression to overt myelofibrosis is 0\u20131% vs. 10\u201312%, and leukemic transformation is 1\u20132% vs. 2\u20136%, in ET and pre-fibrotic-PMF, respectively. Most patients fall in the lower prognostic IPSS group in which observation alone can be recommended. Patients at intermediate risk may require a symptom-driven treatment for anemia, splenomegaly or constitutional symptoms while cytoreductive drugs are indicated in the high-risk category.<\/jats:p>","DOI":"10.1038\/s41408-020-0290-9","type":"journal-article","created":{"date-parts":[[2020,2,25]],"date-time":"2020-02-25T18:02:57Z","timestamp":1582653777000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["The new WHO classification for essential thrombocythemia calls for revision of available evidences"],"prefix":"10.1038","volume":"10","author":[{"given":"Tiziano","family":"Barbui","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"Thiele","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Ferrari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro M.","family":"Vannucchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayalew","family":"Tefferi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,2,25]]},"reference":[{"key":"290_CR1","doi-asserted-by":"publisher","first-page":"937","DOI":"10.1182\/blood-2009-03-209262","volume":"114","author":"JW Vardiman","year":"2009","unstructured":"Vardiman, J. W. et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood 114, 937\u2013951 (2009).","journal-title":"Blood"},{"key":"290_CR2","doi-asserted-by":"publisher","first-page":"2391","DOI":"10.1182\/blood-2016-03-643544","volume":"127","author":"DA Arber","year":"2016","unstructured":"Arber, D. A. et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 127, 2391\u20132405 (2016).","journal-title":"Blood"},{"key":"290_CR3","volume-title":"WHO classification of Tumours of Haematopoietic and Lymphoid Tissues.","author":"SH Swerdlow","year":"2017","unstructured":"Swerdlow, S. H. et al. WHO classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th edn. (International Agency for Research on Cancer, Lyon, France, 2017).","edition":"4th edn."},{"key":"290_CR4","doi-asserted-by":"publisher","first-page":"3227","DOI":"10.1182\/blood-2017-01-761999","volume":"129","author":"P Guglielmelli","year":"2017","unstructured":"Guglielmelli, P. et al. Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis. Blood 129, 3227\u20133236 (2017).","journal-title":"Blood"},{"key":"290_CR5","doi-asserted-by":"publisher","first-page":"101735","DOI":"10.18632\/oncotarget.21594","volume":"8","author":"E Rumi","year":"2017","unstructured":"Rumi, E. et al. Clinical course and outcome of essential thrombocythemia and prefibrotic myelofibrosis according to the revised WHO 2016 diagnostic criteria. Oncotarget 8, 101735\u2013101744 (2017).","journal-title":"Oncotarget"},{"key":"290_CR6","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1080\/17474086.2018.1484280","volume":"11","author":"E Rumi","year":"2018","unstructured":"Rumi, E. et al. Diagnosis and management of prefibrotic myelofibrosis. Expert Rev. Hematol. 11, 537\u2013545 (2018).","journal-title":"Expert Rev. Hematol."},{"key":"290_CR7","doi-asserted-by":"publisher","first-page":"594","DOI":"10.1111\/bjh.14838","volume":"182","author":"M Mudireddy","year":"2018","unstructured":"Mudireddy, M. et al. Prefibrotic versus overtly fibrotic primary myelofibrosis: clinical, cytogenetic, molecular and prognostic comparisons. Br. J. Haematol. 182, 594\u2013597 (2018).","journal-title":"Br. J. Haematol."},{"key":"290_CR8","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1007\/s12185-018-2495-2","volume":"108","author":"A Kamiunten","year":"2018","unstructured":"Kamiunten, A. et al. Early\/prefibrotic primary myelofibrosis in patients who were initially diagnosed with essential thrombocythemia. Int. J. Hematol. 108, 411\u2013415 (2018).","journal-title":"Int. J. Hematol."},{"key":"290_CR9","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1111\/bjh.15562","volume":"183","author":"N Curto-Garcia","year":"2018","unstructured":"Curto-Garcia, N., Ianotto, J. & Harrison, C. N. What is pre-fibrotic myelofibrosis and how should it be managed in 2018? Br. J. Haematol. 183, 23\u201334 (2018).","journal-title":"Br. J. Haematol."},{"key":"290_CR10","doi-asserted-by":"publisher","first-page":"3142","DOI":"10.1182\/blood-2017-04-777805","volume":"129","author":"H Gisslinger","year":"2017","unstructured":"Gisslinger, H. Pre-PMF emerging as important subgroup of MPN. Blood 129, 3142\u20133144 (2017).","journal-title":"Blood"},{"key":"290_CR11","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/j.leukres.2013.09.008","volume":"38","author":"G Barosi","year":"2014","unstructured":"Barosi, G. et al. Identifying and addressing unmet clinical needs in Ph-neg classical myeloproliferative neoplasms: a consensus-based SIE, SIES, GITMO position paper. Leuk. Res. 38, 155\u2013160 (2014).","journal-title":"Leuk. Res."},{"key":"290_CR12","doi-asserted-by":"publisher","first-page":"1126","DOI":"10.1038\/leu.2015.360","volume":"30","author":"H Gisslinger","year":"2016","unstructured":"Gisslinger, H. et al. Clinical impact of bone marrow morphology for the diagnosis of essential thrombocythemia: comparison between the BCSH and the WHO criteria. Leukemia 30, 1126\u20131132 (2016).","journal-title":"Leukemia"},{"key":"290_CR13","doi-asserted-by":"publisher","DOI":"10.1038\/s41408-018-0054-y","volume":"8","author":"T Barbui","year":"2018","unstructured":"Barbui, T. et al. The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion. Blood Cancer J. 8, 15 (2018).","journal-title":"Blood Cancer J."},{"key":"290_CR14","doi-asserted-by":"publisher","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. Blood Cancer J. 5, e366 (2015).","journal-title":"Blood Cancer J."},{"key":"290_CR15","first-page":"29","volume":"34","author":"S Murphy","year":"1997","unstructured":"Murphy, S., Peterson, P., Iland, H. & Laszlo, J. Experience of the Polycythemia Vera Study Group with essential thrombocythemia: a final report on diagnostic criteria, survival, and leukemic transition by treatment. Semin. Hematol. 34, 29\u201339 (1997).","journal-title":"Semin. Hematol."},{"key":"290_CR16","doi-asserted-by":"publisher","first-page":"161","DOI":"10.1111\/j.1365-2796.2004.01357.x","volume":"256","author":"P Johansson","year":"2004","unstructured":"Johansson, P. et al. Trends in the incidence of chronic Philadelphia chromosome negative (Ph-) myeloproliferative disorders in the city of G\u00f6teborg, Sweden, during 1983\u201399. J. Intern. Med. 256, 161\u2013165 (2004).","journal-title":"J. Intern. Med."},{"key":"290_CR17","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1111\/ejh.12873","volume":"98","author":"K Abdulkarim","year":"2017","unstructured":"Abdulkarim, K., Samuelsson, J., Johansson, P. & Andr\u00e9asson, B. Risk factors for vascular complications and treatment patterns at diagnosis of 2389 PV and ET patients: real-world data from the Swedish MPN Registry. Eur. J. Haematol. 98, 577\u2013583 (2017).","journal-title":"Eur. J. Haematol."},{"key":"290_CR18","doi-asserted-by":"publisher","first-page":"5710","DOI":"10.1182\/blood-2010-07-293761","volume":"117","author":"J Thiele","year":"2011","unstructured":"Thiele, J. et al. Essential thrombocythemia versus early primary myelofibrosis: a multicenter study to validate the WHO classification. Blood 117, 5710\u20135718 (2011).","journal-title":"Blood"},{"key":"290_CR19","doi-asserted-by":"publisher","first-page":"885","DOI":"10.1002\/ajh.24788","volume":"92","author":"G Jeryczynski","year":"2017","unstructured":"Jeryczynski, G. et al. Pre-fibrotic\/early primary myelofibrosis vs. WHO-defined essential thrombocythemia: the impact of minor clinical diagnostic criteria on the outcome of the disease. Am. J. Hematol. 92, 885\u2013891 (2017).","journal-title":"Am. J. Hematol."},{"key":"290_CR20","doi-asserted-by":"publisher","first-page":"3179","DOI":"10.1200\/JCO.2010.34.5298","volume":"29","author":"T Barbui","year":"2011","unstructured":"Barbui, T. et al. Survival and disease progression in essential thrombocythemia are significantly influenced by accurate morphologic diagnosis: an international study. J. Clin. Oncol. 29, 3179\u20133184 (2011).","journal-title":"J. Clin. Oncol."},{"key":"290_CR21","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1186\/s13000-015-0269-1","volume":"10","author":"S Rupoli","year":"2015","unstructured":"Rupoli, S. et al. Thrombosis in essential thrombocytemia and early\/prefibrotic primary myelofibrosis: the role of the WHO histological diagnosis. Diagn. Pathol. 10, 29 (2015).","journal-title":"Diagn. Pathol."},{"key":"290_CR22","first-page":"1128","volume":"90","author":"J Thiele","year":"2015","unstructured":"Thiele, J. et al. European consensus on grading bone marrow fibrosis and assessment of cellularity. Haematologica 90, 1128\u20131132 (2015).","journal-title":"Haematologica"},{"key":"290_CR23","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1111\/his.12871","volume":"68","author":"HM Kvasnicka","year":"2016","unstructured":"Kvasnicka, H. M. et al. Problems and pitfalls in grading of bone marrow fibrosis, collagen deposition and osteosclerosis\u2013a consensus-based study. Histopathology 68, 905\u2013915 (2016).","journal-title":"Histopathology"},{"key":"290_CR24","doi-asserted-by":"publisher","first-page":"453","DOI":"10.1016\/j.blre.2016.06.001","volume":"30","author":"T Barbui","year":"2016","unstructured":"Barbui, T. et al. The 2016 revision of WHO classification of myeloproliferative neoplasms: clinical and molecular advances. Blood Rev. 30, 453\u2013459 (2016).","journal-title":"Blood Rev."},{"key":"290_CR25","doi-asserted-by":"publisher","first-page":"2507","DOI":"10.1182\/blood-2014-05-579136","volume":"124","author":"A Tefferi","year":"2014","unstructured":"Tefferi, A. et al. Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. Blood 124, 2507\u20132513 (2014).","journal-title":"Blood"},{"key":"290_CR26","doi-asserted-by":"publisher","first-page":"133","DOI":"10.1002\/ajh.25303","volume":"94","author":"A Tefferi","year":"2019","unstructured":"Tefferi, A. & Barbui, T. Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management. Am. J. Hematol. 94, 133\u2013143 (2019).","journal-title":"Am. J. Hematol."},{"key":"290_CR27","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1002\/ajh.24338","volume":"91","author":"YC Elala","year":"2016","unstructured":"Elala, Y. C. et al. Calreticulin variant stratified driver mutational status and prognosis in essential thrombocythemia. Am. J. Hematol. 91, 503\u2013506 (2016).","journal-title":"Am. J. Hematol."},{"key":"290_CR28","doi-asserted-by":"publisher","first-page":"844","DOI":"10.1182\/blood-2008-01-135897","volume":"112","author":"AM Vannucchi","year":"2008","unstructured":"Vannucchi, A. M. et al. Characteristics and clinical correlates of MPL 515W>L\/K mutation in essential thrombocythemia. Blood 112, 844\u2013847 (2008).","journal-title":"Blood"},{"key":"290_CR29","doi-asserted-by":"publisher","DOI":"10.1038\/s41408-018-0159-3","volume":"8","author":"N Szuber","year":"2018","unstructured":"Szuber, N. et al. MPL-mutated essential thrombocythemia: a morphologic reappraisal. Blood Cancer J. 8, 121 (2018).","journal-title":"Blood Cancer J."},{"key":"290_CR30","doi-asserted-by":"publisher","DOI":"10.1038\/bcj.2016.98","volume":"6","author":"M Haider","year":"2016","unstructured":"Haider, M., Elala, Y. C., Gangat, N., Hanson, C. A. & Tefferi, A. MPL mutations and palpable splenomegaly are independent risk factors for fibrotic progression in essential thrombocythemia. Blood Cancer J. 6, e487 (2016).","journal-title":"Blood Cancer J."},{"key":"290_CR31","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1182\/blood-2008-01-131664","volume":"112","author":"PA Beer","year":"2008","unstructured":"Beer, P. A. et al. MPL mutations in myeloproliferative disorders: analysis of the PT-1 cohort. Blood 112, 141\u2013149 (2008).","journal-title":"Blood"},{"key":"290_CR32","doi-asserted-by":"publisher","first-page":"1551","DOI":"10.1002\/ajh.25230","volume":"93","author":"A Tefferi","year":"2018","unstructured":"Tefferi, A. Primary myelofibrosis: 2019 update on diagnosis, risk-stratification and management. Am. J. Hematol. 93, 1551\u20131560 (2018).","journal-title":"Am. J. Hematol."},{"key":"290_CR33","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1111\/bjh.14202","volume":"179","author":"V Buxhofer-Ausch","year":"2017","unstructured":"Buxhofer-Ausch, V. et al. Impact of white blood cell counts at diagnosis and during follow-up in patients with essential thrombocythaemia and prefibrotic primary myelofibrosis. Br. J. Haematol. 179, 166\u2013169 (2017).","journal-title":"Br. J. Haematol."},{"key":"290_CR34","doi-asserted-by":"publisher","first-page":"5857","DOI":"10.1182\/blood-2011-02-339002","volume":"117","author":"A Carobbio","year":"2011","unstructured":"Carobbio, A. et al. Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: an international study of 891 patients. Blood 117, 5857\u20135859 (2011).","journal-title":"Blood"},{"key":"290_CR35","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1056\/NEJMoa043800","volume":"353","author":"CN Harrison","year":"2005","unstructured":"Harrison, C. N. et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N. Engl. J. Med. 353, 33\u201345 (2005).","journal-title":"N. Engl. J. Med."},{"key":"290_CR36","doi-asserted-by":"publisher","first-page":"2310","DOI":"10.1182\/blood-2006-09-046342","volume":"109","author":"A Carobbio","year":"2007","unstructured":"Carobbio, A. et al. Leukocytosis is a risk factor for thrombosis in essential thrombocythemia: interaction with treatment, standard risk factors, and Jak2 mutation status. Blood 109, 2310\u20132313 (2007).","journal-title":"Blood"},{"key":"290_CR37","doi-asserted-by":"publisher","first-page":"1645","DOI":"10.3324\/haematol.13346","volume":"93","author":"F Passamonti","year":"2008","unstructured":"Passamonti, F. et al. Prognostic factors for thrombosis, myelofibrosis, and leukemia in essential thrombocythemia: a study of 605 patients. Haematologica 93, 1645\u20131651 (2008).","journal-title":"Haematologica"},{"key":"290_CR38","doi-asserted-by":"publisher","first-page":"669","DOI":"10.1002\/ajh.23217","volume":"87","author":"V Buxhofer-Ausch","year":"2012","unstructured":"Buxhofer-Ausch, V. et al. Leukocytosis as an important risk factor for arterial thrombosis in WHO-defined early\/prefibrotic myelofibrosis: an international study of 264 patients. Am. J. Hematol. 87, 669\u2013672 (2012).","journal-title":"Am. J. Hematol."},{"key":"290_CR39","doi-asserted-by":"publisher","first-page":"716","DOI":"10.1038\/leu.2011.258","volume":"26","author":"G Finazzi","year":"2012","unstructured":"Finazzi, G. et al. Incidence and risk factors for bleeding in 1104 patients with essential thrombocythemia or prefibrotic myelofibrosis diagnosed according to the 2008 WHO criteria. Leukemia 26, 716\u2013719 (2012).","journal-title":"Leukemia"},{"key":"290_CR40","doi-asserted-by":"publisher","first-page":"351","DOI":"10.1111\/j.1365-2141.2007.06807.x","volume":"139","author":"DJ Kuter","year":"2007","unstructured":"Kuter, D. J., Bain, B., Mufti, G., Bagg, A. & Hasserjian, R. P. Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres. Br. J. Haematol. 139, 351\u2013362 (2007).","journal-title":"Br. J. Haematol."},{"key":"290_CR41","doi-asserted-by":"publisher","first-page":"2991","DOI":"10.1200\/JCO.2008.20.3174","volume":"27","author":"PJ Campbell","year":"2009","unstructured":"Campbell, P. J. et al. Reticulin accumulation in essential thrombocythemia: prognostic significance and relationship to therapy. J. Clin. Oncol. 27, 2991\u20132999 (2009).","journal-title":"J. Clin. Oncol."},{"key":"290_CR42","doi-asserted-by":"publisher","first-page":"352","DOI":"10.1111\/j.1365-2141.2010.08122.x","volume":"149","author":"CN Harrison","year":"2010","unstructured":"Harrison, C. N. et al. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br. J. Haematol. 149, 352\u2013375 (2010).","journal-title":"Br. J. Haematol."},{"issue":"34","key":"290_CR43","doi-asserted-by":"publisher","first-page":"3361","DOI":"10.1200\/JCO.2018.78.8414","volume":"36","author":"Anna L. Godfrey","year":"2018","unstructured":"Godfrey, A. L. et al. Hydroxycarbamide plus aspirin versus aspirin alone in patients with essential thrombocythemia age 40 to 59 years without high-risk features. J. Clin. Oncol. 36, JCO2018788414 (2018).","journal-title":"Journal of Clinical Oncology"},{"key":"290_CR44","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1182\/blood-2007-05-091850","volume":"111","author":"BS Wilkins","year":"2008","unstructured":"Wilkins, B. S. et al. Bone marrow pathology in essential thrombocythemia: interobserver reliability and utility for identifying disease subtypes. Blood 111, 60\u201370 (2008).","journal-title":"Blood"},{"key":"290_CR45","doi-asserted-by":"publisher","first-page":"1474","DOI":"10.1002\/ajh.25270","volume":"93","author":"N Szuber","year":"2018","unstructured":"Szuber, N. et al. Myeloproliferative neoplasms in the young: Mayo Clinic experience with 361 patients age 40 years or younger. Am. J. Hematol. 93, 1474\u20131484 (2018).","journal-title":"Am. J. Hematol."},{"key":"290_CR46","doi-asserted-by":"publisher","first-page":"362","DOI":"10.1055\/s-2006-942757","volume":"32","author":"HM Kvasnicka","year":"2006","unstructured":"Kvasnicka, H. M. & Thiele, J. The impact of clinicopathological studies on staging and survival in essential thrombocythemia, chronic idiopathic myelofibrosis, and polycythemia rubra vera. Semin. Thromb. Hemost. 32, 362\u2013371 (2006).","journal-title":"Semin. Thromb. Hemost."},{"key":"290_CR47","doi-asserted-by":"publisher","first-page":"148","DOI":"10.1007\/s00277-002-0604-y","volume":"82","author":"J Thiele","year":"2003","unstructured":"Thiele, J. & Kvasnicka, H. M. Chronic myeloproliferative disorders with thrombocythemia: a comparative study of two classification systems (PVSG, WHO) on 839 patients. Ann. Hematol. 82, 148\u2013152 (2003).","journal-title":"Ann. Hematol."},{"key":"290_CR48","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1182\/bloodadvances.2016000208","volume":"1","author":"A Tefferi","year":"2016","unstructured":"Tefferi, A. et al. Targeted deep sequencing in primary myelofibrosis. Blood Adv. 1, 105\u2013111 (2016).","journal-title":"Blood Adv."},{"key":"290_CR49","doi-asserted-by":"publisher","first-page":"1193","DOI":"10.1002\/ajh.24882","volume":"92","author":"A Tefferi","year":"2017","unstructured":"Tefferi, A. et al. Gender and survival in essential thrombocythemia: a two-center study of 1,494 patients. Am. J. Hematol. 92, 1193\u20131197 (2017).","journal-title":"Am. J. Hematol."},{"key":"290_CR50","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1002\/ajh.24320","volume":"91","author":"T Barbui","year":"2016","unstructured":"Barbui, T. Refining prognostication of thrombosis in ET. Am. J. Hematol. 91, 361\u2013363 (2016).","journal-title":"Am. J. Hematol."},{"key":"290_CR51","doi-asserted-by":"publisher","DOI":"10.1038\/s41408-018-0142-z","volume":"8","author":"G Finazzi","year":"2018","unstructured":"Finazzi, G., Vannucchi, A. M. & Barbui, T. Prefibrotic myelofibrosis: treatment algorithm 2018. Blood Cancer J. 8, 104 (2018).","journal-title":"Blood Cancer J."},{"key":"290_CR52","doi-asserted-by":"publisher","DOI":"10.1038\/bcj.2015.94","volume":"5","author":"T Barbui","year":"2015","unstructured":"Barbui, T. et al. Practice-relevant revision of IPSET-thrombosis based on 1019 patients with WHO-defined essential thrombocythemia. Blood Cancer J. 5, e 369 (2015).","journal-title":"Blood Cancer J."},{"key":"290_CR53","doi-asserted-by":"publisher","first-page":"390","DOI":"10.1002\/ajh.24293","volume":"91","author":"M Haider","year":"2016","unstructured":"Haider, M. et al. Validation of the revised international prognostic score of thrombosis for essential thrombocythemia (IPSET-thrombosis) in 585 Mayo Clinic patients. Am. J. Hematol. 91, 390\u2013394 (2016).","journal-title":"Am. J. Hematol."},{"key":"290_CR54","doi-asserted-by":"publisher","first-page":"1057","DOI":"10.1038\/s41375-018-0077-1","volume":"32","author":"T Barbui","year":"2018","unstructured":"Barbui, T. et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia 32, 1057\u20131069 (2018).","journal-title":"Leukemia"},{"key":"290_CR55","doi-asserted-by":"publisher","first-page":"1720","DOI":"10.1182\/blood-2012-07-443770","volume":"121","author":"H Gisslinger","year":"2013","unstructured":"Gisslinger, H. et al. Anagrelide compared with hydroxyurea in WHO-classified essential thrombocythemia: the ANAHYDRET Study, a randomized controlled trial. Blood 121, 1720\u20131728 (2013).","journal-title":"Blood"},{"key":"290_CR56","doi-asserted-by":"publisher","first-page":"51","DOI":"10.3324\/haematol.2017.174672","volume":"103","author":"G Birgeg\u00e5rd","year":"2018","unstructured":"Birgeg\u00e5rd, G. et al. Treatment of essential thrombocythemia in Europe: a prospective long-term observational study of 3649 high-risk patients in the evaluation of anagrelide efficacy and long-term safety study. Haematologica 103, 51\u201360 (2018).","journal-title":"Haematologica"}],"container-title":["Blood Cancer Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/s41408-020-0290-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41408-020-0290-9","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41408-020-0290-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,12,5]],"date-time":"2022-12-05T14:25:33Z","timestamp":1670250333000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/s41408-020-0290-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,2,25]]},"references-count":56,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2020,2]]}},"alternative-id":["290"],"URL":"https:\/\/doi.org\/10.1038\/s41408-020-0290-9","relation":{},"ISSN":["2044-5385"],"issn-type":[{"value":"2044-5385","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,2,25]]},"assertion":[{"value":"16 April 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 May 2019","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 June 2019","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 February 2020","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare that they have no conflict of interest.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}],"article-number":"22"}}