{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T18:11:08Z","timestamp":1787508668258,"version":"build-2736575974"},"reference-count":146,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2019,9,19]],"date-time":"2019-09-19T00:00:00Z","timestamp":1568851200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["1R01-HL116329"],"award-info":[{"award-number":["1R01-HL116329"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005010","name":"Associazione Italiana per la Ricerca sul Cancro","doi-asserted-by":"publisher","award":["AIRC17608"],"award-info":[{"award-number":["AIRC17608"]}],"id":[{"id":"10.13039\/501100005010","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["P01-CA108671"],"award-info":[{"award-number":["P01-CA108671"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"<ns4:p>Myelofibrosis is the advanced stage of the Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), characterized by systemic inflammation, hematopoietic failure in the bone marrow, and development of extramedullary hematopoiesis, mainly in the spleen. The only potentially curative therapy for this disease is hematopoietic stem cell transplantation, an option that may be offered only to those patients with a compatible donor and with an age and functional status that may face its toxicity. By contrast, with the Philadelphia-positive MPNs that can be dramatically modified by inhibitors of the novel BCR-ABL fusion-protein generated by its genetic lesion, the identification of the molecular lesions that lead to the development of myelofibrosis has not yet translated into a treatment that can modify the natural history of the disease. Therefore, the cure of myelofibrosis remains an unmet clinical need. However, the excitement raised by the discovery of the genetic lesions has inspired additional studies aimed at elucidating the mechanisms driving these neoplasms towards their final stage. These studies have generated the feeling that the cure of myelofibrosis will require targeting both the malignant stem cell clone and its supportive microenvironment. We will summarize here some of the biochemical alterations recently identified in MPNs and the novel therapeutic approaches currently under investigation inspired by these discoveries.<\/ns4:p>","DOI":"10.12688\/f1000research.18581.1","type":"journal-article","created":{"date-parts":[[2019,9,19]],"date-time":"2019-09-19T11:30:06Z","timestamp":1568892606000},"page":"1662","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":14,"title":["Novel strategies for the treatment of myelofibrosis driven by recent advances in understanding the role of the microenvironment in its etiology"],"prefix":"10.12688","volume":"8","author":[{"given":"Zimran","family":"Eran","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Zingariello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9299-9189","authenticated-orcid":false,"given":"Maria Teresa","family":"Bochicchio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Bardelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1800-271X","authenticated-orcid":false,"given":"Anna 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Oncol."},{"key":"ref-22","doi-asserted-by":"publisher","first-page":"2233-42","DOI":"10.1182\/blood-2018-12-890889","article-title":"Comprehensive clinical-molecular transplant scoring system for myelofibrosis undergoing stem cell transplantation.","volume":"133","author":"N Gagelmann","year":"2019","journal-title":"Blood."},{"key":"ref-23","doi-asserted-by":"publisher","first-page":"406-14","DOI":"10.1007\/s11899-017-0402-1","article-title":"Contemporary Use of Interferon Therapy in the Myeloproliferative Neoplasms.","volume":"12","author":"C Foucar","year":"2017","journal-title":"Curr Hematol Malig Rep."},{"key":"ref-24","doi-asserted-by":"publisher","first-page":"783-91","DOI":"10.1111\/bjh.12459","article-title":"Efficacy and safety of pegylated-interferon \u03b1-2a in myelofibrosis: A study by the FIM and GEM French cooperative groups.","volume":"162","author":"J Ianotto","year":"2013","journal-title":"Br J 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Cervantes","year":"2017","journal-title":"Blood."},{"key":"ref-34","doi-asserted-by":"publisher","first-page":"1057-69","DOI":"10.1038\/s41375-018-0077-1","article-title":"Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet.","volume":"32","author":"T Barbui","year":"2018","journal-title":"Leukemia."},{"key":"ref-35","doi-asserted-by":"publisher","first-page":"e225-e236","DOI":"10.1016\/S2352-3026(17)30027-3","article-title":"Pacritinib versus best available therapy for the treatment of myelofibrosis irrespective of baseline cytopenias (PERSIST-1): An international, randomised, phase 3 trial.","volume":"4","author":"R Mesa","year":"2017","journal-title":"Lancet Haematol."},{"key":"ref-36","doi-asserted-by":"publisher","first-page":"652-9","DOI":"10.1001\/jamaoncol.2017.5818","article-title":"Pacritinib vs Best Available Therapy, Including Ruxolitinib, in Patients With Myelofibrosis: A Randomized Clinical 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and Advanced Phase Myelofibrosis.","volume":"22","author":"A Keyzner","year":"2016","journal-title":"Biol Blood Marrow Transplant."},{"key":"ref-49","first-page":"199","article-title":"Long-Term Survival in Myelofibrosis after Allogeneic Hematopoietic Cell Transplantation Using Fludarabine\/Melphalan Conditioning Regimen.","volume":"130","author":"H Ali","year":"2017","journal-title":"Blood."},{"key":"ref-50","doi-asserted-by":"crossref","first-page":"1126","DOI":"10.1182\/blood.V128.22.1126.1126","article-title":"Exploring the Potential of JAK1\/2 Inhibitor Ruxolitinib with Reduced Intensity Hematopoietic Cell Transplantation (HCT) for Myelofibrosis: Stage I Results of a Prospective Trial Conducted through the Myeloproliferative Disorders - Research Consortium (MPD-RC).","volume":"128","author":"H Gupta","year":"2016","journal-title":"Blood."},{"key":"ref-51","doi-asserted-by":"publisher","first-page":"5264-70","DOI":"10.1182\/blood-2009-07-234880","article-title":"Allogeneic stem 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growth factor-\u03b2 superfamily ligand trap ACE-536 corrects anemia by promoting late-stage erythropoiesis.","volume":"20","author":"R Suragani","year":"2014","journal-title":"Nat Med."},{"key":"ref-61","doi-asserted-by":"publisher","first-page":"155-166.e17","DOI":"10.1016\/j.exphem.2012.12.002","article-title":"Stromal cell-mediated inhibition of erythropoiesis can be attenuated by Sotatercept (ACE-011), an activin receptor type II ligand trap.","volume":"41","author":"C Iancu-Rubin","year":"2013","journal-title":"Exp Hematol."},{"key":"ref-62","doi-asserted-by":"publisher","first-page":"e63-e72","DOI":"10.1016\/S2352-3026(18)30002-4","article-title":"Sotatercept with long-term extension for the treatment of anaemia in patients with lower-risk myelodysplastic syndromes: a phase 2, dose-ranging trial.","volume":"5","author":"R Komrokji","year":"2018","journal-title":"Lancet 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status","group":{"name":"current-referee-status","label":"Current Referee Status"}},{"value":"10.5256\/f1000research.20338.r53832, Giovanni Martinelli, Maria Teresa Bochicchio, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy, 19 Sep 2019, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/8-1662\/v1#referee-response-53832","order":0,"name":"referee-response-53832","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.20338.r53831, Alessandro Vannucchi, Department of Hematology, Center of Research and Innovation of Myeloproliferative Neoplasms, University of Florence, Florence, Italy, 19 Sep 2019, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/8-1662\/v1#referee-response-53831","order":1,"name":"referee-response-53831","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.20338.r47367, Olatoyosi Odenike, Section of Hematology Oncology, Department of Medicine, University of Chicago Comprehensive Cancer Center, The University of Chicago Medicine, Chicago, IL, USA, 19 Sep 2019, version 1, indexed","URL":"https:\/\/f1000research.com\/articles\/8-1662\/v1#referee-response-47367","order":2,"name":"referee-response-47367","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"This study was supported by grants from the National Cancer Institute (P01-CA108671), National Heart, Lung, and Blood Institute (1R01-HL116329) and Associazione Italiana Ricerca Cancro (AIRC 17608). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.","order":3,"name":"grant-information","label":"Grant Information"},{"value":"This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.","order":0,"name":"copyright-info","label":"Copyright"}]}}