{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T17:26:52Z","timestamp":1789925212276,"version":"4.0.1"},"reference-count":163,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2025,9,27]],"date-time":"2025-09-27T00:00:00Z","timestamp":1758931200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81700130"],"award-info":[{"award-number":["81700130"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Jiangsu Province College Students\u2019 Innovation and Innovation Training Program Project","award":["X2025103120165"],"award-info":[{"award-number":["X2025103120165"]}]},{"name":"Construction Project of High Level Hospital of Jiangsu Province","award":["LCZX202512"],"award-info":[{"award-number":["LCZX202512"]}]},{"name":"Construction Project of High Level Hospital of Jiangsu Province","award":["GSPJS202417"],"award-info":[{"award-number":["GSPJS202417"]}]},{"name":"Construction Project of High Level Hospital of Jiangsu Province","award":["GSPJS202420"],"award-info":[{"award-number":["GSPJS202420"]}]},{"name":"Construction Project of High Level Hospital of Jiangsu Province","award":["GSPJS202414"],"award-info":[{"award-number":["GSPJS202414"]}]},{"name":"Open Project of the Key Laboratory of Thrombosis and Hemostasis of the National Health Commission","award":["KJS2419"],"award-info":[{"award-number":["KJS2419"]}]}],"content-domain":{"domain":["www.mdpi.com"],"crossmark-restriction":true},"short-container-title":["Cancers"],"abstract":"<jats:p>Myeloproliferative neoplasms (MPNs) encompass three principal subtypes: polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). These hematologic malignancies originate from clonal hematopoietic stem cells (HSCs) and exhibit pathological overproduction of myeloid lineage cells. Recent advances in molecular diagnostics, particularly the precise detection of core driver mutations (JAK2 V617F, CALR, and MPL) and non-driver mutations (ASXL1, TET2, SRSF2), has refined diagnostic precision and risk stratification. A variety of prognostic models for MPNs provide guidance for treatment. Treatment methods mainly include bloodletting therapy, low-dose aspirin anticoagulant therapy, cytoreductive therapy, and allogeneic hematopoietic stem cell transplantation (HSCT). JAK inhibitors (such as ruxolitinib) remain the basic therapeutic drugs. However, emerging strategies targeting epigenetic dysregulation and the interaction in the immune microenvironment (such as interferon-\u03b1) show promise in reducing drug resistance. New methods, including combination therapy (combination of JAK inhibitors and BCL-XL inhibitors) and mutation-independent immunotherapy, are under investigation. This review summarizes the latest advancements in the diagnosis and treatment of MPNs, highlighting the importance of molecular mechanisms in guiding therapeutic approaches and the potential for precision medicine in the future.<\/jats:p>","DOI":"10.3390\/cancers17193142","type":"journal-article","created":{"date-parts":[[2025,9,29]],"date-time":"2025-09-29T11:40:52Z","timestamp":1759146052000},"page":"3142","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Advances in the Diagnosis and Treatment of Myeloproliferative Neoplasms (MPNs)"],"prefix":"10.3390","volume":"17","author":[{"given":"Xinyu","family":"Ma","sequence":"first","affiliation":[{"name":"Department of Hematology, The Second Affiliated Hospital of Nanjing Medical University, Nanjing 210003, China"},{"name":"The Second Clinical Medical School, Nanjing Medical University, Nanjing 210011, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhibo","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Hematology, The Second Affiliated Hospital of Nanjing Medical University, Nanjing 210003, China"},{"name":"The Second Clinical Medical School, Nanjing Medical University, Nanjing 210011, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuyu","family":"Gu","sequence":"additional","affiliation":[{"name":"Department of Hematology, The Second Affiliated Hospital of Nanjing Medical University, Nanjing 210003, China"},{"name":"The Second Clinical Medical School, Nanjing Medical University, Nanjing 210011, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yan","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Hematology, The Second Affiliated Hospital of Nanjing Medical University, Nanjing 210003, China"},{"name":"The Second Clinical Medical School, Nanjing Medical University, Nanjing 210011, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tianqing","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Hematology, The Second Affiliated Hospital of Nanjing Medical University, Nanjing 210003, China"},{"name":"The Second Clinical Medical School, Nanjing Medical University, Nanjing 210011, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruonan","family":"Shao","sequence":"additional","affiliation":[{"name":"Department of Hematology, The Second Affiliated Hospital of Nanjing Medical University, Nanjing 210003, China"},{"name":"The Second Clinical Medical School, Nanjing Medical University, Nanjing 210011, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4109-2602","authenticated-orcid":false,"given":"Jinsong","family":"Yan","sequence":"additional","affiliation":[{"name":"Department of Hematology, Liaoning Medical Center for Hematopoietic Stem Cell Transplantation, The Second Hospital of Dalian Medical University, Dalian 116027, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5480-6577","authenticated-orcid":false,"given":"Wei","family":"Chen","sequence":"additional","affiliation":[{"name":"Key Laboratory of Bone Marrow Stem Cells, Department of Hematology, Blood Diseases Institute, Affiliated Hospital of Xuzhou Medical University, No. 99, Western Huaihai Road, Xuzhou 221004, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8547-711X","authenticated-orcid":false,"given":"Xiaofeng","family":"Shi","sequence":"additional","affiliation":[{"name":"Department of Hematology, The Second Affiliated Hospital of Nanjing Medical University, Nanjing 210003, China"},{"name":"The Second Clinical Medical School, Nanjing Medical University, Nanjing 210011, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,9,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Verma, T., Papadantonakis, N., Peker Barclift, D., and Zhang, L. (2024). Molecular Genetic Profile of Myelofibrosis: Implications in the Diagnosis, Prognosis, and Treatment Advancements. Cancers, 16.","DOI":"10.3390\/cancers16030514"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1922","DOI":"10.1182\/blood.2022017416","article-title":"Biology and therapeutic targeting of molecular mechanisms in MPNs","volume":"141","author":"How","year":"2023","journal-title":"Blood"},{"key":"ref_3","first-page":"26","article-title":"How I diagnose and treat essential thrombocythemia","volume":"44","author":"Zhang","year":"2023","journal-title":"Zhonghua Xue Ye Xue Za Zhi"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"102435","DOI":"10.1016\/j.ctrv.2022.102435","article-title":"Impact of molecular profiling on the management of patients with myelofibrosis","volume":"109","author":"Ferrer","year":"2022","journal-title":"Cancer Treat. Rev."},{"key":"ref_5","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_6","doi-asserted-by":"crossref","unstructured":"Baumeister, J., Chatain, N., Sofias, A.M., Lammers, T., and Koschmieder, S. (2021). Progression of Myeloproliferative Neoplasms (MPN): Diagnostic and Therapeutic Perspectives. Cells, 10.","DOI":"10.3390\/cells10123551"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"2851","DOI":"10.1038\/leu.2017.268","article-title":"Application of current prognostic models for primary myelofibrosis in the setting of post-polycythemia vera or post-essential thrombocythemia myelofibrosis","volume":"31","author":"Tefferi","year":"2017","journal-title":"Leukemia"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1182\/blood-2015-03-631747","article-title":"Clonal hematopoiesis of indeterminate potential and its distinction from myelodysplastic syndromes","volume":"126","author":"Steensma","year":"2015","journal-title":"Blood"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2883","DOI":"10.1007\/s11845-024-03776-5","article-title":"Association between JAK2(V617F) variable allele frequency and risk of thrombotic events in patients with myeloproliferative neoplasms","volume":"193","author":"Brown","year":"2024","journal-title":"Ir. J. Med. Sci."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"3115","DOI":"10.1182\/blood-2023-182624","article-title":"Genetic Evolution from Chronic Myeloproliferative Neoplasms to Acute Myeloid Leukemia: An Analysis of Forty-Six Paired Samples","volume":"142","author":"Chang","year":"2023","journal-title":"Blood"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1038\/s41392-021-00791-1","article-title":"The JAK\/STAT signaling pathway: From bench to clinic","volume":"6","author":"Hu","year":"2021","journal-title":"Signal Transduct. Target. Ther."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Kj\u00e6r, L., Skov, V., Larsen, M.K., Boklund, T.I., Andersen, M., Kefala, M., Knudsen, T.A., Schjellerup Eickhardt-Dalb\u00f8ge, C., Stiehl, T., and Gudmand-H\u00f8yer, J. (2023). Case Report: First longitudinal study of a patient with CALR positive clonal hematopoiesis of indeterminate potential developing into pre-fibrotic myelofibrosis. Front. Oncol., 13.","DOI":"10.3389\/fonc.2023.1176173"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1089\/gtmb.2017.0203","article-title":"Development of a Targeted Next-Generation Sequencing Assay to Detect Diagnostically Relevant Mutations of JAK2, CALR, and MPL in Myeloproliferative Neoplasms","volume":"22","author":"Frawley","year":"2018","journal-title":"Genet. Test. Mol. Biomark."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e38089","DOI":"10.7554\/eLife.38089","article-title":"Receptor-mediated dimerization of JAK2 FERM domains is required for JAK2 activation","volume":"7","author":"Ferrao","year":"2018","journal-title":"eLife"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1182\/blood-2016-10-695940","article-title":"Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms","volume":"129","author":"Vainchenker","year":"2017","journal-title":"Blood"},{"key":"ref_16","first-page":"1608","article-title":"Cytokines and Myeloproliferative Neoplasms: Current Research Status from Mechanism to Clinic--Review","volume":"32","author":"Tu","year":"2024","journal-title":"Zhongguo Shi Yan Xue Ye Xue Za Zhi"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2391","DOI":"10.1056\/NEJMoa1312542","article-title":"Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2","volume":"369","author":"Nangalia","year":"2013","journal-title":"N. Engl. J. Med."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Wallace, L., and Obeng, E.A. (2023). Noncoding rules of survival: Epigenetic regulation of normal and malignant hematopoiesis. Front. Mol. Biosci., 10.","DOI":"10.3389\/fmolb.2023.1273046"},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Yu, S., Han, R., and Gan, R. (2022). The Wnt\/\u03b2-catenin signalling pathway in Haematological Neoplasms. Biomark. Res., 10.","DOI":"10.1186\/s40364-022-00418-9"},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Wang, Y., Shtylla, B., and Chou, T. (2024). Order-of-Mutation Effects on Cancer Progression: Models for Myeloproliferative Neoplasm. Bull. Math. Biol., 86.","DOI":"10.1007\/s11538-024-01257-5"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1016\/j.ccr.2012.06.032","article-title":"ASXL1 mutations promote myeloid transformation through loss of PRC2-mediated gene repression","volume":"22","author":"Adli","year":"2012","journal-title":"Cancer Cell"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1186\/s13045-020-00937-8","article-title":"EZH2: A novel target for cancer treatment","volume":"13","author":"Duan","year":"2020","journal-title":"J. Hematol. Oncol."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Testa, U., Pelosi, E., Castelli, G., and Labbaye, C. (2017). miR-146 and miR-155: Two Key Modulators of Immune Response and Tumor Development. Noncoding RNA, 3.","DOI":"10.3390\/ncrna3030022"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1158\/2643-3230.BCD-21-0226","article-title":"PU.1-Dependent Enhancer Inhibition Separates Tet2-Deficient Hematopoiesis from Malignant Transformation","volume":"3","author":"Aivalioti","year":"2022","journal-title":"Blood Cancer Discov."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"e154092","DOI":"10.1172\/JCI154092","article-title":"TGF-\u03b2 signaling in myeloproliferative neoplasms contributes to myelofibrosis without disrupting the hematopoietic niche","volume":"132","author":"Yao","year":"2022","journal-title":"J. Clin. Investig."},{"key":"ref_26","doi-asserted-by":"crossref","unstructured":"Mahmud, M., Vasireddy, S., Gowin, K., and Amaraneni, A. (2023). Myeloproliferative Neoplasms: Contemporary Review and Molecular Landscape. Int. J. Mol. Sci., 24.","DOI":"10.3390\/ijms242417383"},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Dusa, A., Mouton, C., Pecquet, C., Herman, M., and Constantinescu, S.N. (2010). JAK2 V617F constitutive activation requires JH2 residue F595: A pseudokinase domain target for specific inhibitors. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0011157"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1186\/s13045-021-01116-z","article-title":"Molecular pathogenesis of the myeloproliferative neoplasms","volume":"14","author":"Greenfield","year":"2021","journal-title":"J. Hematol. Oncol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"2270","DOI":"10.1210\/me.2007-0111","article-title":"SH2B1 enhances leptin signaling by both Janus kinase 2 Tyr813 phosphorylation-dependent and -independent mechanisms","volume":"21","author":"Li","year":"2007","journal-title":"Mol. Endocrinol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1186\/s12943-023-01827-6","article-title":"PI3K\/AKT\/mTOR signaling transduction pathway and targeted therapies in cancer","volume":"22","author":"Glaviano","year":"2023","journal-title":"Mol. Cancer"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1168","DOI":"10.4161\/cc.8.8.8147","article-title":"How ERK1\/2 activation controls cell proliferation and cell death: Is subcellular localization the answer?","volume":"8","author":"Mebratu","year":"2009","journal-title":"Cell Cycle"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"97","DOI":"10.4251\/wjgo.v5.i5.97","article-title":"RAS signaling pathways, mutations and their role in colorectal cancer","volume":"5","author":"Zenonos","year":"2013","journal-title":"World J. Gastrointest. Oncol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/s41392-024-01934-w","article-title":"The JAK-STAT pathway: From structural biology to cytokine engineering","volume":"9","author":"Lv","year":"2024","journal-title":"Signal Transduct. Target. Ther."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1933","DOI":"10.1158\/1078-0432.CCR-12-0284","article-title":"Molecular pathways: Jak\/STAT pathway: Mutations, inhibitors, and resistance","volume":"19","author":"Verstovsek","year":"2013","journal-title":"Clin. Cancer Res."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1016\/j.apsb.2015.05.007","article-title":"HIF-1\u03b1 pathway: Role, regulation and intervention for cancer therapy","volume":"5","author":"Masoud","year":"2015","journal-title":"Acta Pharm. Sin. B"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1186\/s12967-023-04486-9","article-title":"RAS signaling and immune cells: A sinister crosstalk in the tumor microenvironment","volume":"21","author":"Liu","year":"2023","journal-title":"J. Transl. Med."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"a034876","DOI":"10.1101\/cshperspect.a034876","article-title":"The Molecular Genetics of Myeloproliferative Neoplasms","volume":"10","author":"Marneth","year":"2020","journal-title":"Cold Spring Harb. Perspect. Med."},{"key":"ref_38","first-page":"e17854","article-title":"JAK2 and TET2 Mutation in Polycythemia Vera","volume":"13","author":"Padda","year":"2021","journal-title":"Cureus"},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Rumi, E., Trotti, C., Vanni, D., Casetti, I.C., Pietra, D., and Sant\u2019Antonio, E. (2020). The Genetic Basis of Primary Myelofibrosis and Its Clinical Relevance. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21238885"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41408-023-00969-6","article-title":"Genomic and functional impact of Trp53 inactivation in JAK2V617F myeloproliferative neoplasms","volume":"14","author":"Gou","year":"2024","journal-title":"Blood Cancer J."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1097\/PPO.0000000000000013","article-title":"Genetics of myeloproliferative neoplasms","volume":"20","author":"Viny","year":"2014","journal-title":"Cancer J."},{"key":"ref_42","doi-asserted-by":"crossref","unstructured":"Brune, M.M., Rau, A., Overkamp, M., Flaadt, T., Bonzheim, I., Sch\u00fcrch, C.M., Federmann, B., Dirnhofer, S., Fend, F., and Tzankov, A. (2021). Molecular Progression of Myeloproliferative and Myelodysplastic\/Myeloproliferative Neoplasms: A Study on Sequential Bone Marrow Biopsies. Cancers, 13.","DOI":"10.3390\/cancers13225605"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1038\/s41375-023-01886-0","article-title":"Clonal architecture evolution in Myeloproliferative Neoplasms: From a driver mutation to a complex heterogeneous mutational and phenotypic landscape","volume":"37","author":"Maslah","year":"2023","journal-title":"Leukemia"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1182\/bloodadvances.2020003444","article-title":"Genomic analysis of primary and secondary myelofibrosis redefines the prognostic impact of ASXL1 mutations: A FIM study","volume":"5","author":"Riou","year":"2021","journal-title":"Blood Adv."},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Irino, T., Uemura, M., Yamane, H., Umemura, S., Utsumi, T., Kakazu, N., Shirakawa, T., Ito, M., Suzuki, T., and Kinoshita, K. (2011). JAK2 V617F-dependent upregulation of PU.1 expression in the peripheral blood of myeloproliferative neoplasm patients. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0022148"},{"key":"ref_46","doi-asserted-by":"crossref","unstructured":"Aujla, A., Linder, K., Iragavarapu, C., Karass, M., and Liu, D. (2018). SRSF2 mutations in myelodysplasia\/myeloproliferative neoplasms. Biomark. Res., 6.","DOI":"10.1186\/s40364-018-0142-y"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1531","DOI":"10.1038\/s41588-023-01480-1","article-title":"Single-cell multi-omics identifies chronic inflammation as a driver of TP53-mutant leukemic evolution","volume":"55","author":"Norfo","year":"2023","journal-title":"Nat. Genet."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/j.blre.2016.06.001","article-title":"The 2016 revision of WHO classification of myeloproliferative neoplasms: Clinical and molecular advances","volume":"30","author":"Barbui","year":"2016","journal-title":"Blood Rev."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1038\/s41375-022-01613-1","article-title":"The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic\/Dendritic Neoplasms","volume":"36","author":"Khoury","year":"2022","journal-title":"Leukemia"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"5907","DOI":"10.1007\/s12010-022-04051-y","article-title":"Development of a Real-Time qPCR Assay for Detection of Common MPL Mutations in Myeloproliferative Neoplasms (MPNS)","volume":"194","author":"Ullah","year":"2022","journal-title":"Appl. Biochem. Biotechnol."},{"key":"ref_51","doi-asserted-by":"crossref","unstructured":"Skov, V. (2020). Next Generation Sequencing in MPNs. Lessons from the Past and Prospects for Use as Predictors of Prognosis and Treatment Responses. Cancers, 12.","DOI":"10.3390\/cancers12082194"},{"key":"ref_52","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_53","doi-asserted-by":"crossref","first-page":"1465","DOI":"10.1002\/ajh.27002","article-title":"Polycythemia vera: 2024 update on diagnosis, risk-stratification, and management","volume":"98","author":"Tefferi","year":"2023","journal-title":"Am. J. Hematol."},{"key":"ref_54","first-page":"62","article-title":"Congenital and acquired polycythemias","volume":"105","author":"Siegel","year":"2008","journal-title":"Dtsch. Arztebl. Int."},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"Iurlo, A., Cattaneo, D., Bucelli, C., and Baldini, L. (2020). New Perspectives on Polycythemia Vera: From Diagnosis to Therapy. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21165805"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1002\/ajh.27216","article-title":"Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management","volume":"99","author":"Tefferi","year":"2024","journal-title":"Am. J. Hematol."},{"key":"ref_57","first-page":"911","article-title":"Value of bone marrow biopsy in the diagnosis of essential thrombocythemia","volume":"89","author":"Florena","year":"2004","journal-title":"Haematologica"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1002\/ajh.25011","article-title":"Chronic myeloid leukemia: 2018 update on diagnosis, therapy and monitoring","volume":"93","author":"Jabbour","year":"2018","journal-title":"Am. J. Hematol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1200","DOI":"10.1182\/blood.2022015850","article-title":"International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: Integrating morphologic, clinical, and genomic data","volume":"140","author":"Arber","year":"2022","journal-title":"Blood"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"2228","DOI":"10.1182\/blood.2022015853","article-title":"Genomic profiling for clinical decision making in myeloid neoplasms and acute leukemia","volume":"140","author":"Duncavage","year":"2022","journal-title":"Blood"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1111\/ijlh.13536","article-title":"Cytomorphology of normal, reactive, dysmorphic, and dysplastic megakaryocytes in bone marrow aspirates","volume":"43","author":"Zini","year":"2021","journal-title":"Int. J. Lab. Hematol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1774","DOI":"10.1080\/10428190600678975","article-title":"Essential thrombocythemia or chronic idiopathic myelofibrosis? A single-center study based on hematopoietic bone marrow histology","volume":"47","author":"Gianelli","year":"2006","journal-title":"Leuk. Lymphoma"},{"key":"ref_63","doi-asserted-by":"crossref","unstructured":"Combaluzier, S., Quessada, J., Abbou, N., Arcani, R., Tichadou, A., Gabert, J., Costello, R., Loosveld, M., Venton, G., and Berda-Haddad, Y. (2023). Cytological Diagnosis of Classic Myeloproliferative Neoplasms at the Age of Molecular Biology. Cells, 12.","DOI":"10.3390\/cells12060946"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1002\/ajh.24396","article-title":"Chronic myelomonocytic leukemia: 2016 update on diagnosis, risk stratification, and management","volume":"91","author":"Patnaik","year":"2016","journal-title":"Am. J. Hematol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1954","DOI":"10.1182\/blood.2022017423","article-title":"Myelofibrosis","volume":"141","author":"Passamonti","year":"2023","journal-title":"Blood"},{"key":"ref_66","unstructured":"Leukemia and Lymphoma Group of the Hematology Society of the Chinese Medical Association (2022). Chinese guideline for the diagnosis and treatment of polycythemia vera (2022). Zhonghua Xue Ye Xue Za Zhi, 43, 537\u2013541."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1111\/bjh.15648","article-title":"A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline","volume":"184","author":"McMullin","year":"2019","journal-title":"Br. J. Haematol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1874","DOI":"10.1038\/leu.2013.163","article-title":"Survival and prognosis among 1545 patients with contemporary polycythemia vera: An international study","volume":"27","author":"Tefferi","year":"2013","journal-title":"Leukemia"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"2726","DOI":"10.1038\/leu.2017.169","article-title":"A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis","volume":"31","author":"Passamonti","year":"2017","journal-title":"Leukemia"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1182\/blood-2012-01-403279","article-title":"A prognostic model to predict survival in 867 World Health Organization-defined essential thrombocythemia at diagnosis: A study by the International Working Group on Myelofibrosis Research and Treatment","volume":"120","author":"Passamonti","year":"2012","journal-title":"Blood"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1472","DOI":"10.1002\/ajh.26332","article-title":"Clinical and molecular predictors of fibrotic progression in essential thrombocythemia: A multicenter study involving 1607 patients","volume":"96","author":"Loscocco","year":"2021","journal-title":"Am. J. Hematol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1111\/bjh.16380","article-title":"Mutation-enhanced international prognostic systems for essential thrombocythaemia and polycythaemia vera","volume":"189","author":"Tefferi","year":"2020","journal-title":"Br. J. Haematol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1729","DOI":"10.1182\/bloodadvances.2019000211","article-title":"Leukocytosis and thrombosis in essential thrombocythemia and polycythemia vera: A systematic review and meta-analysis","volume":"3","author":"Carobbio","year":"2019","journal-title":"Blood Adv."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"e369","DOI":"10.1038\/bcj.2015.94","article-title":"Practice-relevant revision of IPSET-thrombosis based on 1019 patients with WHO-defined essential thrombocythemia","volume":"5","author":"Barbui","year":"2015","journal-title":"Blood Cancer J."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"5857","DOI":"10.1182\/blood-2011-02-339002","article-title":"Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: An international study of 891 patients","volume":"117","author":"Carobbio","year":"2011","journal-title":"Blood"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"5128","DOI":"10.1182\/blood-2012-07-444067","article-title":"Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis)","volume":"120","author":"Barbui","year":"2012","journal-title":"Blood"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1111\/ejh.13516","article-title":"Impact of platelets on major thrombosis in patients with a normal white blood cell count in essential thrombocythemia","volume":"106","author":"Wolf","year":"2021","journal-title":"Eur. J. Haematol."},{"key":"ref_78","first-page":"92","article-title":"Clinical evaluation of the revised International Prognostic Score of Thrombosis for essential thrombocythemia (IPSET-thrombosis) in a cohort of 746 Chinese adult patients","volume":"38","author":"Fu","year":"2017","journal-title":"Zhonghua Xue Ye Xue Za Zhi"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"e936","DOI":"10.1097\/HS9.0000000000000936","article-title":"Application of IPSET-thrombosis in 1366 Patients Prospectively Followed From the Spanish Registry of Essential Thrombocythemia","volume":"7","author":"Cuevas","year":"2023","journal-title":"Hemasphere"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1002\/ajh.26857","article-title":"Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management","volume":"98","author":"Tefferi","year":"2023","journal-title":"Am. J. Hematol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.6004\/jnccn.2022.0046","article-title":"Myeloproliferative Neoplasms, Version 3.2022, NCCN Clinical Practice Guidelines in Oncology","volume":"20","author":"Gerds","year":"2022","journal-title":"J. Natl. Compr. Cancer Netw."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"2895","DOI":"10.1182\/blood-2008-07-170449","article-title":"New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment","volume":"113","author":"Cervantes","year":"2009","journal-title":"Blood"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1182\/blood-2009-09-245837","article-title":"A dynamic prognostic model to predict survival in primary myelofibrosis: A study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment)","volume":"115","author":"Passamonti","year":"2010","journal-title":"Blood"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1200\/JCO.2010.32.2446","article-title":"DIPSS plus: A refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status","volume":"29","author":"Gangat","year":"2011","journal-title":"J. Clin. Oncol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1200\/JCO.2017.76.4886","article-title":"MIPSS70: Mutation-Enhanced International Prognostic Score System for Transplantation-Age Patients with Primary Myelofibrosis","volume":"36","author":"Guglielmelli","year":"2018","journal-title":"J. Clin. Oncol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1769","DOI":"10.1200\/JCO.2018.78.9867","article-title":"MIPSS70+ Version 2.0: Mutation and Karyotype-Enhanced International Prognostic Scoring System for Primary Myelofibrosis","volume":"36","author":"Tefferi","year":"2018","journal-title":"J. Clin. Oncol."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1631","DOI":"10.1038\/s41375-018-0107-z","article-title":"GIPSS: Genetically inspired prognostic scoring system for primary myelofibrosis","volume":"32","author":"Tefferi","year":"2018","journal-title":"Leukemia"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1002\/ajh.25335","article-title":"Genetically inspired prognostic scoring system (GIPSS) outperforms dynamic international prognostic scoring system (DIPSS) in myelofibrosis patients","volume":"94","author":"Kuykendall","year":"2019","journal-title":"Am. J. Hematol."},{"key":"ref_89","first-page":"488","article-title":"The case for phlebotomy in polycythemia vera","volume":"32","author":"Dameshek","year":"1968","journal-title":"Blood"},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1080\/03007995.2025.2458531","article-title":"Improving the management of Polycythemia Vera patients eligible for cytoreduction: Report of a multidisciplinary advisory board","volume":"41","author":"Ramundo","year":"2025","journal-title":"Curr. Med. Res. Opin."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1001\/jama.2024.20377","article-title":"Diagnosis and Treatment of Polycythemia Vera: A Review","volume":"333","author":"Tremblay","year":"2025","journal-title":"JAMA"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1056\/NEJMoa1208500","article-title":"Cardiovascular events and intensity of treatment in polycythemia vera","volume":"368","author":"Marchioli","year":"2013","journal-title":"N. Engl. J. Med."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/j.blre.2012.06.002","article-title":"Front-line therapy in polycythemia vera and essential thrombocythemia","volume":"26","author":"Barbui","year":"2012","journal-title":"Blood Rev."},{"key":"ref_94","first-page":"30","article-title":"Influence of therapy on causes of death in polycythemia vera","volume":"94","author":"Wasserman","year":"1981","journal-title":"Trans. Assoc. Am. Physicians"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1080\/09537100600758677","article-title":"Platelet-mediated erythromelalgic, cerebral, ocular and coronary microvascular ischemic and thrombotic manifestations in patients with essential thrombocythemia and polycythemia vera: A distinct aspirin-responsive and coumadin-resistant arterial thrombophilia","volume":"17","author":"Michiels","year":"2006","journal-title":"Platelets"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1002\/ajh.21730","article-title":"Polycythemia vera-associated acquired von Willebrand syndrome despite near-normal platelet count","volume":"85","author":"Tefferi","year":"2010","journal-title":"Am. J. Hematol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1182\/blood.V6.4.372.372","article-title":"Some speculations on the myeloproliferative syndromes","volume":"6","author":"Dameshek","year":"1951","journal-title":"Blood"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1111\/j.1365-2141.2004.05277.x","article-title":"Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia","volume":"128","author":"Elliott","year":"2005","journal-title":"Br. J. Haematol."},{"key":"ref_99","first-page":"17","article-title":"From efficacy to safety: A Polycythemia Vera Study group report on hydroxyurea in patients with polycythemia vera","volume":"34","author":"Fruchtman","year":"1997","journal-title":"Semin. Hematol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1038\/leu.2014.250","article-title":"Clinical end points for drug treatment trials in BCR-ABL1-negative classic myeloproliferative neoplasms: Consensus statements from European LeukemiaNET (ELN) and Internation Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT)","volume":"29","author":"Barosi","year":"2015","journal-title":"Leukemia"},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1007\/s00277-023-05089-6","article-title":"Real-world treatments and thrombotic events in polycythemia vera patients in the USA","volume":"102","author":"Verstovsek","year":"2023","journal-title":"Ann. Hematol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"106809","DOI":"10.1016\/j.leukres.2022.106809","article-title":"Thrombotic events and mortality risk in patients with newly diagnosed polycythemia vera or essential thrombocythemia","volume":"115","author":"Pemmaraju","year":"2022","journal-title":"Leuk. Res."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"3065","DOI":"10.1182\/blood-2008-03-143537","article-title":"Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera","volume":"112","author":"Kiladjian","year":"2008","journal-title":"Blood"},{"key":"ref_104","first-page":"40","article-title":"Interferon alfa: Effects of long-term treatment for polycythemia vera","volume":"34","author":"Silver","year":"1997","journal-title":"Semin. Hematol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"2397","DOI":"10.1002\/cncr.21900","article-title":"A phase II trial of pegylated interferon alpha-2b therapy for polycythemia vera and essential thrombocythemia: Feasibility, clinical and biologic effects, and impact on quality of life","volume":"106","author":"Samuelsson","year":"2006","journal-title":"Cancer"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1002\/cncr.22026","article-title":"Long-term effects of the treatment of polycythemia vera with recombinant interferon-alpha","volume":"107","author":"Silver","year":"2006","journal-title":"Cancer"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"5418","DOI":"10.1200\/JCO.2009.23.6075","article-title":"Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera","volume":"27","author":"Kantarjian","year":"2009","journal-title":"J. Clin. Oncol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"1643","DOI":"10.1038\/s41375-020-01020-4","article-title":"Interferon alpha therapy in essential thrombocythemia and polycythemia vera-a systematic review and meta-analysis","volume":"35","author":"Bewersdorf","year":"2021","journal-title":"Leukemia"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"e473912","DOI":"10.1200\/EDBK-25-473912","article-title":"Pegylated Interferons: Still a Major Player for the Treatment of Myeloproliferative Neoplasms","volume":"45","author":"Daunov","year":"2025","journal-title":"Am. Soc. Clin. Oncol. Educ. Book."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/s13045-017-0395-1","article-title":"Can pegylated interferon improve the outcome of polycythemia vera patients?","volume":"10","author":"Cerrano","year":"2017","journal-title":"J. Hematol. Oncol."},{"key":"ref_111","first-page":"262","article-title":"[Clinical Value of Detecting ABL Kinase Domain Mutations in Patients with Chronic Myeloid Leukemia Based on High-Throughput Sequencing Technology]","volume":"32","author":"Zhou","year":"2024","journal-title":"Zhongguo Shi Yan Xue Ye Xue Za Zhi"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1002\/ajh.27311","article-title":"Novel germline JAK2(R715T) mutation causing PV-like erythrocytosis in 3 generations. Amelioration by Ropeg-Interferon","volume":"99","author":"Song","year":"2024","journal-title":"Am. J. Hematol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1016\/S1470-2045(16)30558-7","article-title":"Ruxolitinib for the treatment of inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): A randomised, open-label, phase 3b study","volume":"18","author":"Passamonti","year":"2017","journal-title":"Lancet Oncol."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"3534","DOI":"10.1200\/JCO.22.01935","article-title":"Ruxolitinib Versus Best Available Therapy for Polycythemia Vera Intolerant or Resistant to Hydroxycarbamide in a Randomized Trial","volume":"41","author":"Harrison","year":"2023","journal-title":"J. Clin. Oncol."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"2037","DOI":"10.1007\/s00277-014-2152-7","article-title":"Busulfan in patients with polycythemia vera or essential thrombocythemia refractory or intolerant to hydroxyurea","volume":"93","author":"Antelo","year":"2014","journal-title":"Ann. Hematol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1080\/10428194.2016.1187269","article-title":"Busulfan is effective second-line therapy for older patients with Philadelphia-negative myeloproliferative neoplasms intolerant of or unresponsive to hydroxyurea","volume":"58","author":"Douglas","year":"2017","journal-title":"Leuk. Lymphoma"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"3179","DOI":"10.1200\/JCO.2010.34.5298","article-title":"Survival and disease progression in essential thrombocythemia are significantly influenced by accurate morphologic diagnosis: An international study","volume":"29","author":"Barbui","year":"2011","journal-title":"J. Clin. Oncol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"2664","DOI":"10.1182\/blood-2004-09-3426","article-title":"Acute leukemia in polycythemia vera: An analysis of 1638 patients enrolled in a prospective observational study","volume":"105","author":"Finazzi","year":"2005","journal-title":"Blood"},{"key":"ref_119","doi-asserted-by":"crossref","unstructured":"Yadav, R., Hakobyan, N., and Wang, J.C. (2023). Role of Next Generation Immune Checkpoint Inhibitor (ICI) Therapy in Philadelphia Negative Classic Myeloproliferative Neoplasm (MPN): Review of the Literature. Int. J. Mol. Sci., 24.","DOI":"10.20944\/preprints202307.0136.v1"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1080\/10428194.2021.1992756","article-title":"Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States","volume":"63","author":"Verstovsek","year":"2022","journal-title":"Leuk. Lymphoma"},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1111\/ejh.12256","article-title":"Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union","volume":"92","author":"Moulard","year":"2014","journal-title":"Eur. J. Haematol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1111\/j.1365-2141.2010.08122.x","article-title":"Guideline for investigation and management of adults and children presenting with a thrombocytosis","volume":"149","author":"Harrison","year":"2010","journal-title":"Br. J. Haematol."},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1038\/s41375-018-0077-1","article-title":"Philadelphia chromosome-negative classical myeloproliferative neoplasms: Revised management recommendations from European LeukemiaNet","volume":"32","author":"Barbui","year":"2018","journal-title":"Leukemia"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"1943","DOI":"10.1182\/blood.2022017625","article-title":"Essential thrombocythemia: Challenges in clinical practice and future prospects","volume":"141","author":"Godfrey","year":"2023","journal-title":"Blood"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"926","DOI":"10.3324\/haematol.2016.146654","article-title":"Antiplatelet therapy versus observation in low-risk essential thrombocythemia with a CALR mutation","volume":"101","author":"Pereira","year":"2016","journal-title":"Haematologica"},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1182\/blood.2019004596","article-title":"A randomized double-blind trial of 3 aspirin regimens to optimize antiplatelet therapy in essential thrombocythemia","volume":"136","author":"Rocca","year":"2020","journal-title":"Blood"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"2619","DOI":"10.1161\/CIR.0b013e318202f701","article-title":"ACCF\/ACG\/AHA 2010 Expert Consensus Document on the concomitant use of proton pump inhibitors and thienopyridines: A focused update of the ACCF\/ACG\/AHA 2008 expert consensus document on reducing the gastrointestinal risks of antiplatelet therapy and NSAID use: A report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents","volume":"122","author":"Abraham","year":"2010","journal-title":"Circulation"},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"1945","DOI":"10.1016\/S0140-6736(05)67785-9","article-title":"Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: A prospective study","volume":"366","author":"Campbell","year":"2005","journal-title":"Lancet"},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"S300","DOI":"10.1016\/j.clml.2013.05.015","article-title":"Successful long-term treatment of Philadelphia chromosome-negative myeloproliferative neoplasms with combination of hydroxyurea and anagrelide","volume":"13","author":"Ahn","year":"2013","journal-title":"Clin. Lymphoma Myeloma Leuk."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"2216","DOI":"10.1080\/10428190802353625","article-title":"The combined use of hydroxyurea and anagrelide allows satisfactory hematologic control in patients with chronic myeloproliferative disorders and thrombocytosis: A report on 13 patients with poor tolerance to hydroxyurea monotherapy","volume":"49","author":"Micheletti","year":"2008","journal-title":"Leuk. Lymphoma"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"679","DOI":"10.3324\/haematol.2012.083097","article-title":"Combination therapy of hydroxycarbamide with anagrelide in patients with essential thrombocythemia in the evaluation of Xagrid(R) efficacy and long-term safety study","volume":"99","author":"Gugliotta","year":"2014","journal-title":"Haematologica"},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"2931","DOI":"10.1182\/blood.2021012743","article-title":"A randomized phase 3 trial of interferon-\u03b1 vs hydroxyurea in polycythemia vera and essential thrombocythemia","volume":"139","author":"Mascarenhas","year":"2022","journal-title":"Blood"},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"4551","DOI":"10.1158\/1078-0432.CCR-22-1128","article-title":"Inhibition of LSD1 with Bomedemstat Sensitizes Small Cell Lung Cancer to Immune Checkpoint Blockade and T-Cell Killing","volume":"28","author":"Hiatt","year":"2022","journal-title":"Clin. Cancer Res."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1080\/13543784.2023.2267980","article-title":"Bomedemstat as an investigative treatment for myeloproliferative neoplasms","volume":"32","author":"Rienhoff","year":"2023","journal-title":"Expert. Opin. Investig. Drugs"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1056\/NEJMoa1503479","article-title":"Telomerase Inhibitor Imetelstat in Patients with Essential Thrombocythemia","volume":"373","author":"Baerlocher","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"2701","DOI":"10.1182\/blood.2023020731","article-title":"The role of the MDM2\/p53 axis in antitumor immune responses","volume":"143","author":"Brummer","year":"2024","journal-title":"Blood"},{"key":"ref_137","doi-asserted-by":"crossref","unstructured":"Shallis, R.M., and Podoltsev, N.A. (2021). Emerging agents and regimens for polycythemia vera and essential thrombocythemia. Biomark. Res., 9.","DOI":"10.1186\/s40364-021-00298-5"},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"1532","DOI":"10.1002\/ajh.26349","article-title":"2021 Update on allogeneic hematopoietic stem cell transplant for myelofibrosis: A review of current data and applications on risk stratification and management","volume":"96","author":"Ali","year":"2021","journal-title":"Am. J. Hematol."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"1464","DOI":"10.1002\/ajh.26654","article-title":"Innovative strategies to improve hematopoietic stem cell transplant outcomes in myelofibrosis","volume":"97","author":"Perram","year":"2022","journal-title":"Am. J. Hematol."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1002\/ajh.26279","article-title":"Allogeneic hematopoietic cell transplantation in older myelofibrosis patients: A study of the chronic malignancies working party of EBMT and the Spanish Myelofibrosis Registry","volume":"96","author":"Pereira","year":"2021","journal-title":"Am. J. Hematol."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1002\/ajh.26034","article-title":"Splenectomy before allogeneic hematopoietic cell transplantation for myelofibrosis: A French nationwide study","volume":"96","author":"Bossard","year":"2021","journal-title":"Am. J. Hematol."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1016\/j.bbmt.2017.03.002","article-title":"The Impact of Splenectomy in Myelofibrosis Patients before Allogeneic Hematopoietic Stem Cell Transplantation","volume":"23","author":"Robin","year":"2017","journal-title":"Biol. Blood Marrow Transpl."},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1002\/ajh.26020","article-title":"Impact of spleen size and splenectomy on outcomes of allogeneic hematopoietic cell transplantation for myelofibrosis: A retrospective analysis by the chronic malignancies working party on behalf of European society for blood and marrow transplantation (EBMT)","volume":"96","author":"Polverelli","year":"2021","journal-title":"Am. J. Hematol."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"3551","DOI":"10.1038\/s41375-021-01276-4","article-title":"Impact of prior JAK-inhibitor therapy with ruxolitinib on outcome after allogeneic hematopoietic stem cell transplantation for myelofibrosis: A study of the CMWP of EBMT","volume":"35","author":"Sbianchi","year":"2021","journal-title":"Leukemia"},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"1888","DOI":"10.1038\/s41409-021-01252-7","article-title":"Ruxolitinib before allogeneic hematopoietic transplantation in patients with myelofibrosis on behalf SFGM-TC and FIM groups","volume":"56","author":"Robin","year":"2021","journal-title":"Bone Marrow Transpl."},{"key":"ref_146","first-page":"327","article-title":"Agnogenic myeloid metaplasia","volume":"22","author":"Tefferi","year":"1995","journal-title":"Semin. Oncol."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1097\/PPO.0b013e31815a7c0a","article-title":"New and old treatment modalities in primary myelofibrosis","volume":"13","author":"Cervantes","year":"2007","journal-title":"Cancer J."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1007\/s00277-010-1019-9","article-title":"Efficacy and tolerability of hydroxyurea in the treatment of the hyperproliferative manifestations of myelofibrosis: Results in 40 patients","volume":"89","author":"Gaya","year":"2010","journal-title":"Ann. Hematol."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1056\/NEJMoa1110556","article-title":"JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis","volume":"366","author":"Harrison","year":"2012","journal-title":"N. Engl. J. Med."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"2226","DOI":"10.1182\/blood.V95.7.2226","article-title":"Splenectomy in myelofibrosis with myeloid metaplasia: A single-institution experience with 223 patients","volume":"95","author":"Tefferi","year":"2000","journal-title":"Blood"},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"E235","DOI":"10.1002\/ajh.25203","article-title":"Early thrombotic events and preemptive systemic anticoagulation following splenectomy for myelofibrosis","volume":"93","author":"Mudireddy","year":"2018","journal-title":"Am. J. Hematol."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1111\/bjh.13262","article-title":"Momelotinib treatment-emergent neuropathy: Prevalence, risk factors and outcome in 100 patients with myelofibrosis","volume":"169","author":"Abdelrahman","year":"2015","journal-title":"Br. J. Haematol."},{"key":"ref_153","doi-asserted-by":"crossref","unstructured":"De Almeida, S., Regimbeau, M., Jego, G., Garrido, C., Girodon, F., and Hermetet, F. (2020). Heat Shock Proteins and PD-1\/PD-L1 as Potential Therapeutic Targets in Myeloproliferative Neoplasms. Cancers, 12.","DOI":"10.3390\/cancers12092592"},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"100005","DOI":"10.1016\/j.cytox.2019.100005","article-title":"Cytokines frequently implicated in myeloproliferative neoplasms","volume":"1","author":"Wang","year":"2019","journal-title":"Cytokine X"},{"key":"ref_155","doi-asserted-by":"crossref","unstructured":"Longhitano, L., Li Volti, G., Giallongo, C., Spampinato, M., Barbagallo, I., Di Rosa, M., Romano, A., Avola, R., Tibullo, D., and Palumbo, G.A. (2020). The Role of Inflammation and Inflammasome in Myeloproliferative Disease. J. Clin. Med., 9.","DOI":"10.3390\/jcm9082334"},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1007\/s13402-018-0392-6","article-title":"TLR4 and RAGE conversely mediate pro-inflammatory S100A8\/9-mediated inhibition of proliferation-linked signaling in myeloproliferative neoplasms","volume":"41","author":"Mossuz","year":"2018","journal-title":"Cell. Oncol."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1158\/2643-3230.BCD-22-0176","article-title":"The Role of Inflammation in the Initiation and Progression of Myeloid Neoplasms","volume":"4","author":"Lasry","year":"2023","journal-title":"Blood Cancer Discov."},{"key":"ref_158","doi-asserted-by":"crossref","unstructured":"Fisher, D.A.C., Fowles, J.S., Zhou, A., and Oh, S.T. (2021). Inflammatory Pathophysiology as a Contributor to Myeloproliferative Neoplasms. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.683401"},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"2414","DOI":"10.1182\/blood.2023022804","article-title":"In vivo ablation of NF-\u03baB cascade effectors alleviates disease burden in myeloproliferative neoplasms","volume":"143","author":"Laranjeira","year":"2024","journal-title":"Blood"},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1056\/NEJMoa2308809","article-title":"Rusfertide, a Hepcidin Mimetic, for Control of Erythrocytosis in Polycythemia Vera","volume":"390","author":"Kremyanskaya","year":"2024","journal-title":"N. Engl. J. Med."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"4059","DOI":"10.2217\/fon-2022-0901","article-title":"BOREAS: A global, phase III study of the MDM2 inhibitor navtemadlin (KRT-232) in relapsed\/refractory myelofibrosis","volume":"18","author":"Verstovsek","year":"2022","journal-title":"Future Oncol."},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1016\/j.celrep.2013.10.038","article-title":"Combined targeting of JAK2 and Bcl-2\/Bcl-xL to cure mutant JAK2-driven malignancies and overcome acquired resistance to JAK2 inhibitors","volume":"5","author":"Waibel","year":"2013","journal-title":"Cell Rep."},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"100056","DOI":"10.1016\/j.bneo.2024.100056","article-title":"Addition of navitoclax to ruxolitinib for patients with myelofibrosis with progression or suboptimal response","volume":"2","author":"Pemmaraju","year":"2025","journal-title":"Blood Neoplasia"}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/17\/19\/3142\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,29]],"date-time":"2025-09-29T11:51:55Z","timestamp":1759146715000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/17\/19\/3142"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9,27]]},"references-count":163,"journal-issue":{"issue":"19","published-online":{"date-parts":[[2025,10]]}},"alternative-id":["cancers17193142"],"URL":"https:\/\/doi.org\/10.3390\/cancers17193142","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,9,27]]}}}