JAK inhibition in myelofibrosis: how to sequence treatment in this new era of multiple options

References

• Dameshek W. Some speculations on the myeloproliferative syndromes. Blood. 1951;6(4):372–375.
   PubMed Web of Science ®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.
   PubMed Web of Science ®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):787–798.
   PubMed Web of Science ®Google Scholar
• Verstovsek S, Mesa RA, Gotlib J, COMFORT-I investigators, et al. Long-term treatment with ruxolitinib for patients with myelofibrosis: 5-year update from the randomized, double-blind, placebo-controlled, phase 3 COMFORT-I trial. J Hematol Oncol. 2017;10(1):55.
   PubMedGoogle Scholar
• Guglielmelli P, Ghirardi A, Carobbio A, et al. Impact of ruxolitinib on survival of patients with myelofibrosis in the real world: update of the ERNEST study. Blood Adv. 2022;6(2):373–375.
   PubMed Web of Science ®Google Scholar
• Barosi G, Gale RP. Does ruxolitinib really prolong survival in individuals with myelofibrosis? The never-ending story. Blood Adv. 2022;6(7):2331–2333.
   PubMed Web of Science ®Google Scholar
• Pardanani A, Harrison C, Cortes JE, et al. Safety and efficacy of fedratinib in patients with primary or secondary myelofibrosis: a randomized clinical trial. JAMA Oncol. 2015;1(5):643–651.
   PubMed Web of Science ®Google Scholar
• Harrison CN, Schaap N, Vannucchi AM, et al. Janus kinase-2 inhibitor fedratinib in patients with myelofibrosis previously treated with ruxolitinib (JAKARTA-2): a single-arm, open-label, non-randomised, phase 2, multicentre study. Lancet Haematol. 2017;4(7):e317–e324.
   PubMed Web of Science ®Google Scholar
• Harrison CN, Schaap N, Vannucchi AM, et al. Fedratinib in patients with myelofibrosis previously treated with ruxolitinib: an updated analysis of the JAKARTA2 study using stringent criteria for ruxolitinib failure. Am J Hematol. 2020;95(6):594–603.
   PubMed Web of Science ®Google Scholar
• Gupta VAY, Srdan V, Ruben AM, et al. editor. Safety and tolerability results from the phase 3B freedom trial of fedratinib (fedr), an oral, selective jak2 inhibitor, in patients with myelofibrosis (MF) previously treated with ruxolitinib (rux). European Hematology Association; 2022, Presented at: 2022 EHA Congress; June 9-12, 2022; Vienna, Austria. Abstract P1042.
   Google Scholar
• Venugopal S, Mascarenhas J. The odyssey of pacritinib in myelofibrosis. Blood Adv. 2022 Aug 23;6(16):4905–4913. doi: 10.1182/bloodadvances.2022007524.
   Web of Science ®Google Scholar
• Mesa RA, Vannucchi AM, Mead A, et al. Pacritinib versus best available therapy for the treatment of myelofibrosis irrespective of baseline cytopenias (PERSIST-1): an international, randomised, phase 3 trial. Lancet Haematol. 2017; 4(5):e225–e236.
   PubMed Web of Science ®Google Scholar
• Mascarenhas J, Hoffman R, Talpaz M, et al. Pacritinib vs best available therapy, including ruxolitinib, in patients with myelofibrosis: a randomized clinical trial. JAMA Oncol. 2018;4(5):652–659.
   PubMed Web of Science ®Google Scholar
• Gerds AT, Savona MR, Scott BL, et al. Determining the recommended dose of pacritinib: results from the PAC203 dose-finding trial in advanced myelofibrosis. Blood Adv. 2020;4(22):5825–5835.
   PubMed Web of Science ®Google Scholar
• Pemmaraju N, Scott BL, Savona MR, et al. Risk-adjusted safety analysis of pacritinib (PAC) in patients (pts) with myelofibrosis (MF). J Clin Oncol. 2022;40(16_suppl):7058–7058.
   Web of Science ®Google Scholar
• Mesa RA, Kiladjian JJ, Catalano JV, et al. SIMPLIFY-1: a phase III randomized trial of momelotinib versus ruxolitinib in janus kinase inhibitor-naive patients with myelofibrosis. J Clin Oncol. 2017;35(34):3844–3850.
   PubMed Web of Science ®Google Scholar
• Oh ST, Talpaz M, Gerds AT, et al. ACVR1/JAK1/JAK2 inhibitor momelotinib reverses transfusion dependency and suppresses hepcidin in myelofibrosis phase 2 trial. Blood Adv. 2020;4(18):4282–4291.
   PubMed Web of Science ®Google Scholar
• Asshoff M, Petzer V, Warr MR, et al. Momelotinib inhibits ACVR1/ALK2, decreases hepcidin production, and ameliorates anemia of chronic disease in rodents. Blood. 2017;129(13):1823–1830.
   PubMed Web of Science ®Google Scholar
• Harrison CN, Vannucchi AM, Platzbecker U, et al. Momelotinib versus best available therapy in patients with myelofibrosis previously treated with ruxolitinib (SIMPLIFY 2): a randomised, open-label, phase 3 trial. Lancet Haematol. 2018;5(2):e73–e81.
   PubMed Web of Science ®Google Scholar
• Mesa RA, Gerds AT, Vannucchi A, et al. MOMENTUM: Phase 3 randomized study of momelotinib (MMB) versus danazol (DAN) in symptomatic and anemic myelofibrosis (MF) patients previously treated with a JAK inhibitor. J Clin Oncol. 2022;40(16_suppl):7002–7002.
   Web of Science ®Google Scholar
• NCCN Practice Guidelines Myeloproliferative Neoplasms. 2022. [cited 2022 August 2]. www.nccn.org
   Google Scholar
• Rampal RK, Mascarenhas JO, Kosiorek HE, et al. Safety and efficacy of combined ruxolitinib and decitabine in accelerated and blast-phase myeloproliferative neoplasms. Blood Adv. 2018;2(24):3572–3580.
   PubMed Web of Science ®Google Scholar
• Bose P, Verstovsek S, Cortes JE, et al. A phase 1/2 study of ruxolitinib and decitabine in patients with post-myeloproliferative neoplasm acute myeloid leukemia. Leukemia. 2020;34(9):2489–2492.
   PubMed Web of Science ®Google Scholar
• Mascarenhas JO, Rampal RK, Kosiorek HE, et al. Phase 2 study of ruxolitinib and decitabine in patients with myeloproliferative neoplasm in accelerated and blast phase. Blood Adv. 2020;4(20):5246–5256.
   PubMed Web of Science ®Google Scholar
• Cervantes F, Ross DM, Radinoff A, et al. Efficacy and safety of a novel dosing strategy for ruxolitinib in the treatment of patients with myelofibrosis and anemia: the REALISE phase 2 study. Leukemia. 2021;35(12):3455–3465.
   PubMed Web of Science ®Google Scholar
• Pemmaraju N, Verstovsek S, Mesa R, et al. Defining disease modification in myelofibrosis in the era of targeted therapy. Cancer. 2022;128(13):2420–2432.
   PubMed Web of Science ®Google Scholar