{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T04:53:00Z","timestamp":1760244780713,"version":"build-2065373602"},"reference-count":19,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2022,12,6]],"date-time":"2022-12-06T00:00:00Z","timestamp":1670284800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Introduction: Essential thrombocythemia is a chronic myeloproliferative neoplasm associated with thrombo-hemorrhagic events and the progression to myelofibrosis or acute myeloid leukemia. The purpose of this article is to present real-world data on ET cases diagnosed and managed between 1998 and 2020 in the largest, tertiary hematology reference center in Romania and to evaluate the impact of thrombotic events on survival. Methods: A real-world, retrospective cohort-type study was conducted. We collected and statistically analyzed data from 168 patients who met the 2016 WHO diagnostic criteria for ET and who were managed between 1998 and 2020 in our center. Results: The median age at diagnosis of ET was 51.8 years, with a female predominance (66.07%). The JAK2V617F mutation was detected in 60.71% of patients. Leukocytosis at diagnosis was associated with a higher risk of thrombosis, and JAK2V617F-positive cases exhibited a 1.5-fold higher risk of developing thrombotic events. The average survival in ET with major thrombosis was 14.5 years versus 20.6 years in ET cases without major thrombosis. Other predictors of survival were high-risk IPSET score and age &gt;60 years. Conclusions: Romanian patients diagnosed with ET are generally younger than 60 years and are predominantly female. The occurrence of thrombotic events was influenced by gender, leukocyte count at diagnosis and JAK2V617F positivity. Survival was impacted by age, the presence of JAK2V617F mutation, hypertension, major thrombotic complications and IPSET score. Notably, these findings warrant careful interpretation and further confirmation in the setting of prospective studies.<\/jats:p>","DOI":"10.3390\/medicina58121798","type":"journal-article","created":{"date-parts":[[2022,12,7]],"date-time":"2022-12-07T02:18:48Z","timestamp":1670379528000},"page":"1798","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Essential Thrombocythemia: One-Center Data in a Changing Disease"],"prefix":"10.3390","volume":"58","author":[{"given":"Nicoleta","family":"Pirciulescu","sequence":"first","affiliation":[{"name":"Department of Clinical Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7133-8875","authenticated-orcid":false,"given":"Mihnea-Alexandru","family":"Gaman","sequence":"additional","affiliation":[{"name":"Department of Clinical Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"},{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marina","family":"Mihailescu","sequence":"additional","affiliation":[{"name":"Department of Clinical Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"Constantin","sequence":"additional","affiliation":[{"name":"Department of Clinical Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mihaela","family":"Dragomir","sequence":"additional","affiliation":[{"name":"Department of Molecular Biology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Camelia","family":"Dobrea","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"Department of Hematopathology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simona","family":"Costache","sequence":"additional","affiliation":[{"name":"Department of Cytomorphology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Iulia","family":"Ursuleac","sequence":"additional","affiliation":[{"name":"Department of Clinical Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"},{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Coriu","sequence":"additional","affiliation":[{"name":"Department of Clinical Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"},{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Manuela","family":"Crisan","sequence":"additional","affiliation":[{"name":"Department of Clinical Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, 022328 Bucharest, Romania"},{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,12,6]]},"reference":[{"key":"ref_1","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"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Rungjirajittranon, T., Owattanapanich, W., Ungprasert, P., Siritanaratkul, N., and Ruchutrakool, T. (2019). A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms. BMC Cancer, 19.","DOI":"10.1186\/s12885-019-5387-9"},{"key":"ref_3","first-page":"2634853520978210","article-title":"The Essential Thrombocythemia in 2020: What We Know and Where We Still Have to Dig Deep","volume":"13","author":"Accurso","year":"2020","journal-title":"Clin. Med. Insights: Blood Disord."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Mej\u00eda-Ochoa, M., Acevedo Toro, P.A., and Cardona-Arias, J.A. (2019). Systematization of analytical studies of polycythemia vera, essential thrombocythemia and primary myelofibrosis, and a meta-analysis of the frequency of JAK2, CALR and MPL mutations: 2000\u20132018. BMC Cancer, 19.","DOI":"10.1186\/s12885-019-5764-4"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1054","DOI":"10.1016\/S0140-6736(05)71142-9","article-title":"Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders","volume":"365","author":"Baxter","year":"2005","journal-title":"Lancet"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"291","DOI":"10.3343\/alm.2016.36.4.291","article-title":"Screening PCR versus sanger sequencing: Detection of CALR mutations in patients with thrombocytosis","volume":"36","author":"Jeong","year":"2016","journal-title":"Ann. Lab. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1190","DOI":"10.3904\/kjim.2020.634","article-title":"Thrombotic and hemorrhagic events in 2016 World Health Organization-defined Philadelphia-negative myeloproliferative neoplasm","volume":"36","author":"Song","year":"2021","journal-title":"Korean J. Intern. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1007\/s00277-010-1154-3","article-title":"Impact of leukocytosis on thrombotic risk and survival in 532 patients with essential thrombocythemia: A retrospective study","volume":"90","author":"Palandri","year":"2011","journal-title":"Ann. Hematol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"317","DOI":"10.7326\/M17-0028","article-title":"Risk for Arterial and Venous Thrombosis in Patients With Myeloproliferative Neoplasms: A Population-Based Cohort Study","volume":"168","author":"Hultcrantz","year":"2018","journal-title":"Ann. Intern. Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1016\/j.thromres.2009.02.004","article-title":"Association of V617F Jak2 mutation with the risk of thrombosis among patients with essential thrombocythaemia or idiopathic myelofibrosis: A systematic review","volume":"124","author":"Lussana","year":"2009","journal-title":"Thromb. Res."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1038\/s41408-018-0100-9","article-title":"Splanchnic vein thrombosis in myeloproliferative neoplasms: Treatment algorithm 2018","volume":"8","author":"Finazzi","year":"2018","journal-title":"Blood Cancer J."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1645","DOI":"10.3324\/haematol.13346","article-title":"Prognostic factors for thrombosis, myelofibrosis, and leukemia in essential thrombocythemia: A study of 605 patients","volume":"93","author":"Passamonti","year":"2008","journal-title":"Haematologica"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"3042","DOI":"10.1182\/blood-2018-99-109892","article-title":"Excess Mortality in Polycythemia Vera and Essential Thrombocythemia","volume":"132","author":"Pereira","year":"2018","journal-title":"Blood"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1002\/ajh.24882","article-title":"Gender and survival in essential thrombocythemia: A two-center study of 1,494 patients","volume":"92","author":"Tefferi","year":"2017","journal-title":"Am. J. Hematol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1111\/ejh.12517","article-title":"Predictors of survival and cause of death in patients with essential thrombocythemia","volume":"95","author":"Lekovic","year":"2015","journal-title":"Eur. J. Haematol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"159","DOI":"10.4065\/81.2.159","article-title":"Essential thrombocythemia beyond the first decade: Life expectancy, long-term complication rates, and prognostic factors","volume":"81","author":"Wolanskyj","year":"2006","journal-title":"Mayo Clin. Proc."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1007\/s11239-019-02029-y","article-title":"Risk factors and incidence of thrombosis in a Brazilian cohort of patients with Philadelphia-negative myeloproliferative neoplasms","volume":"49","author":"Seguro","year":"2020","journal-title":"J. Thromb. Thrombolysis"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2507","DOI":"10.1182\/blood-2014-05-579136","article-title":"Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis","volume":"124","author":"Tefferi","year":"2014","journal-title":"Blood"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1344","DOI":"10.1038\/leu.2015.87","article-title":"Mutations and long-term outcome of 217 young patients with essential thrombocythemia or early primary myelofibrosis","volume":"29","author":"Palandri","year":"2015","journal-title":"Leukemia"}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/58\/12\/1798\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:35:12Z","timestamp":1760146512000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/58\/12\/1798"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,6]]},"references-count":19,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["medicina58121798"],"URL":"https:\/\/doi.org\/10.3390\/medicina58121798","relation":{},"ISSN":["1648-9144"],"issn-type":[{"type":"electronic","value":"1648-9144"}],"subject":[],"published":{"date-parts":[[2022,12,6]]}}}