Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391–405. https://doi.org/10.1182/blood-2016-03-643544.

Article  CAS  PubMed  Google Scholar 

Klampfl T, Gisslinger H, Harutyunyan AS, et al. Somatic mutations of calreticulin in myeloproliferative neoplasms. N Engl J Med. 2013;369(25):2379–90. https://doi.org/10.1056/NEJMoa1311347.

Article  CAS  PubMed  Google Scholar 

Guijarro-Hernandez A, Vizmanos JL. A broad overview of signaling in Ph-negative classic myeloproliferative neoplasms. Cancers (Basel). 2021;13(5):984. https://doi.org/10.3390/cancers13050984.

Article  CAS  PubMed  Google Scholar 

Geyer HL, Dueck AC, Scherber RM, Mesa RA. Impact of inflammation on myeloproliferative neoplasm symptom development. Mediators Inflamm. 2015;2015:284706. https://doi.org/10.1155/2015/284706.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Fisher DAC, Fowles JS, Zhou A, Oh ST. Inflammatory pathophysiology as a contributor to myeloproliferative neoplasms. Front Immunol. 2021;12:683401. https://doi.org/10.3389/fimmu.2021.683401.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Tefferi A. Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 2021;96(1):145–62. https://doi.org/10.1002/ajh.26050.

Article  CAS  PubMed  Google Scholar 

Price GL, Davis KL, Karve S, Pohl G, Walgren RA. Survival patterns in United States (US) Medicare enrollees with non-CML myeloproliferative neoplasms (MPN). PLoS ONE. 2014;9(3):e90299. https://doi.org/10.1371/journal.pone.0090299.

Article  ADS  CAS  PubMed  PubMed Central  Google Scholar 

Hultcrantz M, Kristinsson SY, Andersson TM, et al. Patterns of survival among patients with myeloproliferative neoplasms diagnosed in Sweden from 1973 to 2008: a population-based study. J Clin Oncol. 2012;30(24):2995–3001. https://doi.org/10.1200/JCO.2012.42.1925.

Article  PubMed  PubMed Central  Google Scholar 

Mesa R, Miller CB, Thyne M, et al. Myeloproliferative neoplasms (MPNs) have a significant impact on patients’ overall health and productivity: the MPN Landmark survey. BMC Cancer. 2016;16:167. https://doi.org/10.1186/s12885-016-2208-2.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Titmarsh GJ, Duncombe AS, McMullin MF, et al. How common are myeloproliferative neoplasms? A systematic review and meta-analysis. Am J Hematol. 2014;89(6):581–7. https://doi.org/10.1002/ajh.23690.

Article  PubMed  Google Scholar 

Mehta J, Wang H, Iqbal SU, Mesa R. Epidemiology of myeloproliferative neoplasms in the United States. Leuk Lymphoma. 2014;55(3):595–600. https://doi.org/10.3109/10428194.2013.813500.

Article  PubMed  Google Scholar 

National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Myeloproliferative Neoplasms, version 2.2023. Available at: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1477. Accessed October 9, 2023.

JAKAFI® (ruxolitinib). Full Prescribing Information, Incyte Corporation, Wilmington, DE, 2023.

Inrebic® (fedratinib). Full Prescribing Information, Celgene Corporation, Summit, NJ, USA, 2019.

VONJO® (pacritinib). Full Prescribing Information, CTI BioPharma Corporation, Seattle, WA, 2022.

OJJAARA (momelotinib). Full Prescribing Information, GlaxoSmithKline, Durham, NC, 2023.

Al-Ali HK, Griesshammer M, Foltz L, et al. Primary analysis of JUMP, a phase 3b, expanded-access study evaluating the safety and efficacy of ruxolitinib in patients with myelofibrosis, including those with low platelet counts. Br J Haematol. 2020;189(5):888–903. https://doi.org/10.1111/bjh.16462. JUMP is the largest clinical trial of patients with MF,including evaluation of patients with early(intermediate-1 risk)MF.

Article  CAS  PubMed  Google Scholar 

Al-Ali HK, Griesshammer M, le Coutre P, et al. Safety and efficacy of ruxolitinib in an open-label, multicenter, single-arm phase 3b expanded-access study in patients with myelofibrosis: a snapshot of 1144 patients in the JUMP trial. Haematologica. 2016;101(9):1065–73. https://doi.org/10.3324/haematol.2016.143677. JUMP is the largest clinical trial of patients with MF,including evaluation of patients with early(intermediate-1risk) MF.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Mead AJ, Milojkovic D, Knapper S, et al. Response to ruxolitinib in patients with intermediate-1-, intermediate-2-, and high-risk myelofibrosis: results of the UK ROBUST Trial. Br J Haematol. 2015;170(1):29–39. https://doi.org/10.1111/bjh.13379. ROBUST includes evaluation of patients with early(intermediate-1risk) MF.

Article  CAS  PubMed  Google Scholar 

Palandri F, Tiribelli M, Benevolo G, et al. Efficacy and safety of ruxolitinib in intermediate-1 IPSS risk myelofibrosis patients: results from an independent study. Hematol Oncol. 2018;36(1):285–90. https://doi.org/10.1002/hon.2429. Includes evaluation of patients with early(intermediate-1risk) MF.

Article  CAS  PubMed  Google Scholar 

Deininger M, Radich J, Burn TC, Huber R, Paranagama D, Verstovsek S. The effect of long-term ruxolitinib treatment on JAK2p.V617F allele burden in patients with myelofibrosis. Blood. 2015;126(13):1551–4. https://doi.org/10.1182/blood-2015-03-635235.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Harrison C, Kiladjian JJ, Al-Ali HK, et al. JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N Engl J Med. 2012;366(9):787–98. https://doi.org/10.1056/NEJMoa1110556.

Article  CAS  PubMed  Google Scholar 

Palandri F, Palumbo GA, Bonifacio M, et al. Baseline factors associated with response to ruxolitinib: an independent study on 408 patients with myelofibrosis. Oncotarget. 2017;8(45):79073–86. https://doi.org/10.18632/oncotarget.18674.

Article  PubMed  PubMed Central  Google Scholar 

Verstovsek S, Kiladjian JJ, Vannucchi AM, et al. Early intervention in myelofibrosis and impact on outcomes: a pooled analysis of the COMFORT-I and COMFORT-II studies. Cancer. 2023;129(11):1681–90. https://doi.org/10.1002/cncr.34707. Post hoc analysis of pooled data from the COMFORT-I and -II phase 3 studies, indicating treatment with minimal delay is associated with improved clinical outcomes.

Article  CAS  PubMed  Google Scholar 

Verstovsek S, Mesa RA, Gotlib J, et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med. 2012;366(9):799–807. https://doi.org/10.1056/NEJMoa1110557.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Verstovsek S, Gotlib J, Mesa RA, et al. Long-term survival in patients treated with ruxolitinib for myelofibrosis: COMFORT-I and -II pooled analyses. J Hematol Oncol. 2017;10(1):156. https://doi.org/10.1186/s13045-017-0527-7.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Palandri F, Palumbo GA, Bonifacio M, et al. Durability of spleen response affects the outcome of ruxolitinib-treated patients with myelofibrosis: results from a multicentre study on 284 patients. Leuk Res. 2018;74:86–8. https://doi.org/10.1016/j.leukres.2018.10.001.

Article  CAS  PubMed  Google Scholar 

Scherber R, Dueck AC, Johansson P, et al. The Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF): international prospective validation and reliability trial in 402 patients. Blood. 2011;118(2):401–8. https://doi.org/10.1182/blood-2011-01-328955.

Article  CAS  PubMed  Google Scholar 

Verstovsek S, Yu J, Kish JK, et al. Real-world risk assessment and treatment initiation among patients with myelofibrosis at community oncology practices in the United States. Ann Hematol. 2020;99(11):2555–64. https://doi.org/10.1007/s00277-020-04055-w.

Article  PubMed  PubMed Central  Google Scholar 

Guglielmelli P, Lasho TL, Rotunno G, et al. MIPSS70: Mutation-enhanced International Prognostic Score System for transplantation-age patients with primary myelofibrosis. J Clin Oncol. 2018;36(4):310–8. https://doi.org/10.1200/JCO.2017.76.4886.

Article  CAS  PubMed  Google Scholar 

Passamonti F, Giorgino T, Mora B, et al. A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis. Leukemia. 2017;31(12):2726–31. https://doi.org/10.1038/leu.2017.169.

Article  CAS  PubMed  Google Scholar 

Guglielmelli P, Mora B, Gesullo F, et al. JAK2V617F molecular response to ruxolitinib in patients with PV and ET is associated with lower risk of progression to secondary myelofibrosis. Blood. 2022;140(suppl 1):1788–9. https://doi.org/10.1182/blood-2022-156781.

Article  Google Scholar 

Harrison CN, Nangalia J, Boucher R, et al. Ruxolitinib versus best available therapy for polycythemia vera intolerant or resistant to hydroxycarbamide in a randomized trial. J Clin Oncol. 2023;41(19):3534–44. https://doi.org/10.1200/JCO.22.01935.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Gerds AT, Lyons RM, Colucci P, Kalafut P, Paranagama D, Verstovsek S. Disease and clinical characteristics of patients with a clinical diagnosis of myelofibrosis enrolled in the MOST study. Clin Lymphoma Myeloma Leuk. 2022;22(7):e532–40. https://doi.org/10.1016/j.clml.2022.02.001.

Article  CAS  PubMed  Google Scholar 

Komrokji RS, Grunwald MR, Braunstein E, Hamer-Maansson JE, Kalafut P, Mascarenhas J. Disease progression and leukemic transformation in patients with lower-risk myelofibrosis (MF): an analysis from MOST. Blood. 2022;140(suppl 1):6832–4. https://doi.org/10.1182/blood-2022-159181.

Article  Google Scholar 

Pizzi M, Silver RT, Barel A, Orazi A. Recombinant interferon-α in myelofibrosis reduces bone marrow fibrosis, improves its morphology and is associated with clinical response. Mod Pathol. 2015;28(10):1315–23. https://doi.org/10.1038/modpathol.2015.93.

Article  CAS