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2L Therapy for PV and ET

CME

Preparing for the Future: Integration of Second-line Therapies After Hydroxyurea for PV and ET

Physicians: Maximum of 0.25 AMA PRA Category 1 Credit

Released: April 29, 2026

Expiration: October 28, 2026

Activity

Progress
1 2 3
Course Completed

References

  1. ‌National Comprehensive Cancer Network. Clinical practice guidelines in oncology: myeloproliferative neoplasms. v.1.2026. nccn.org. Accessed April 28, 2026.
  2. McMullin MF, Harrison CN, Ali S, et al. A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline. Br J Haematol. 2019;184:176-191.
  3. Marchioli R, Finazzi G, Landolfi R, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. 2005;23:2224-2232.
  4. Mascarenhas J, Kosiorek HE, Prchal JT, et al. A randomized phase 3 trial of interferon-α vs hydroxyurea in polycythemia vera and essential thrombocythemia. Blood. 2022;139:2931-2941.
  5. Kiladjian JJ, Klade C, Georgiev P, et al. Long-term outcomes of polycythemia vera patients treated with ropeginterferon alfa-2b. Leukemia. 2022;36:1408-1411.
  6. Gisslinger H, Klade C, Georgiev P, et al. Event-free survival in patients with polycythemia vera treated with ropeginterferon alfa-2b versus best available treatment. Leukemia. 2023;37:2129-2132.
  7. Barbui T, Vannucchi AM, Finazzi G, et al. A reappraisal of the benefit-risk profile of hydroxyurea in polycythemia vera: A propensity-matched study. Am J Hematol. 2017;92:1131-1136.
  8. Marchioli R, Finazzi G, Specchia G, et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013;368:22-33.
  9. Barosi G, Birgegard G, Finazzi G, et al. A unified definition of clinical resistance and intolerance to hydroxycarbamide in polycythaemia vera and primary myelofibrosis: results of a European LeukemiaNet (ELN) consensus process. Br J Haematol. 2010;148:961-963.
  10. 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;41:3534-3544.
  11. Vannucchi AM, Kiladjian JJ, Griesshammer M, et al. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015;372:426-435.
  12. Kiladjian JJ, Zachee P, Hino M, et al. Long-term efficacy and safety of ruxolitinib versus best available therapy in polycythaemia vera (RESPONSE): 5-year follow up of a phase 3 study. Lancet Haematol. 2020;7:e226-e237.
  13. Barbui T, Vannucchi AM, Buxhofer-Ausch V, et al. Practice-relevant revision of IPSET-thrombosis based on 1019 patients with WHO-defined essential thrombocythemia. Blood Cancer J. 2015;5:e369.
  14. Rotunno G, Mannarelli C, Guglielmelli P, et al. Impact of calreticulin mutations on clinical and hematological phenotype and outcome in essential thrombocythemia. Blood. 2014;123:1552-1555.
  15. Loscocco GG, Gesullo F, Capecchi G, et al. One thousand patients with essential thrombocythemia: the Florence-CRIMM experience. Blood Cancer J. 2024;14:10.
  16. Gangat N, Karrar O, Al-Kali A, et al. One thousand patients with essential thrombocythemia: the Mayo Clinic experience. Blood Cancer J. 2024;14:11.
  17. Hernández-Boluda JC, Alvarez-Larrán A, Gómez M, et al. Clinical evaluation of the European LeukaemiaNet criteria for clinicohaematological response and resistance/intolerance to hydroxycarbamide in essential thrombocythaemia. Br J Haematol. 2010;152:81-88.
  18. ‌Barosi G, Besses C, Birgegard G, et al. A unified definition of clinical resistance/intolerance to hydroxyurea in essential thrombocythemia: results of a consensus process by an international working group. Leukemia. 2007;21:277-280.
  19. Harrison CN, Mead AJ, Panchal A, et al. Ruxolitinib vs best available therapy for ET intolerant or resistant to hydroxycarbamide. Blood. 2017;130:1889-1897.
  20. Mesa R, Gill H, Zhang L, et al. Ropeginterferon alfa-2b in hydroxyurea-intolerant or hydroxyurea-refractory essential thrombocythaemia (SURPASS ET): a multicentre, open-label, randomised, active-controlled, phase 3 study. Lancet Haematology. 2025;12:e862-e875.
  21. ‌Ropeginterferon alfa-2b-njft [prescribing information]. Burlington, MA: PharmaEssentia USA Corporation; 2024.
  22. Harrison CN, Campbell PJ, Buck G, et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005;353:33-45.
  23. ‌Kremyanskaya M, Kuykendall AT, Pemmaraju N, et al. Rusfertide, a hepcidin mimetic, for control of erythrocytosis in polycythemia vera. N Engl J Med. 2024;390:723-735.
  24. Kuykendall AT, Pemmaraju N, Pettit KM, et al. Results from VERIFY, a phase 3, double-blind, placebo (PBO)-controlled study of rusfertide for treatment of polycythemia vera (PV). Presented at: 2025 American Society of Clinical Oncology Annual Conference; May 30 - June 3, 2025. Abstract LBA3.
  25. Kuykendall A, Bankar A, Pettit K, et al. Rusfertide or placebo plus current standard-of-care therapy for polycythemia vera: Durability of response and safety results through week 52 from the randomized controlled phase 3 VERIFY study. Presented at: 2025 American Society of Hematology Annual Conference. December 6-9, 2025. Abstract 81.
  26. Niebel D, Kirfel J, Janzen V, et al. Lysine-specific demethylase 1 (LSD1) in hematopoietic and lymphoid neoplasms. Blood. 2014;124:151-152.
  27. Jutzi JS, Kleppe M, Dias J, et al. LSD1 inhibition prolongs survival in mouse models of MPN by selectively targeting the disease clone. HemaSphere. 2018;2:e54.
  28. Gill H. Lysine-Specific demethylase 1 (LSD1/KDM1A) inhibition as a target for disease modification in myelofibrosis. Cells. 2022;11:2107.
  29. Goethert JR, Gill H, Palandri F, et al. Bomedemstat (IMG-7289), an LSD1 inhibitor, manages the signs and symptoms of essential thrombocythemia (ET) while reducing the burden of cells homozygous for driver mutations. Presented at: 2023 American Society of Hematology Annual Conference; December 9-12, 2023. Abstract 747.
  30. Rein L, Livings A, Frewin R, et al. Efficacy and safety of the LSD1 inhibitor bomedemstat in participants with polycythemia vera (PV) resistant or intolerant to cytoreductive therapy: the phase 2 shorespan-004 study. Presented at: 2025 American Society of Hematology Annual Conference; December 6-9, 2025. Abstract 83.
  31. Landolfi R, Di Gennaro L, Barbui T, et al. Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood. 2007;109:2446-2452.
  32. Barbui T, Ghirardi A, Carobbio A, et al. Thrombosis in myeloproliferative neoplasms: a viewpoint on its impact on myelofibrosis, mortality, and solid tumors. Blood Cancer J. 2024;14:188.
  33. Passamonti F, Patriarca A, Knapper S, et al. Pelabresib (CPI-0610) monotherapy in high-risk essential thrombocythemia refractory or intolerant to hydroxyurea: preliminary results from MANIFEST study. Presented at: 2023 American Society of Clinical Oncology Annual Conference; June 2-6, 2023. Abstract 7019.
  34. Gupta V, Mascarenhas J, Ali H, et al. Safety and efficacy of INCA033989, a novel first in class mutant calreticulin-specific monoclonal antibody, in patients with essential thrombocythemia. Presented at: 67th ASH Annual Meeting and Exposition; December 6-9, 2025. Abstract 1024.
  35. Kuchnio A, Samakai E, Hug E, et al. Discovery of JNJ-88549968, a novel, first-in-class CALRmutxCD3 T-Cell redirecting antibody for the treatment of myeloproliferative neoplasms. Presented at: 65th ASH Annual Meeting and Exposition; December 9-12, 2023. Abstract 1777.
  36. Gotlib J, Mascarenhas J, Rampal R, et al. A multicenter, open-label phase 1 study of INCB160058, a first-in-class JAK2V617F mutant–selective inhibitor, in patients with myelofibrosis, polycythemia vera, or essential thrombocythemia. Presented at: 67th ASH Annual Meeting and Exposition; December 6-9, 2025. Abstract 2051.