Ask AI
Risk Stratification in ET and PV

CME

Risk Stratification in Polycythemia Vera and Essential Thrombocythemia: Applying Prognostic Scoring Systems in Practice

Physicians: Maximum of 0.25 AMA PRA Category 1 Credit

Released: February 03, 2026

Expiration: August 02, 2026

Activity

Progress
1 2
Course Completed

References

  1. Tefferi A, Barbui T. Polycythemia vera: 2024 update on diagnosis, risk-stratification, and management. Am J Hematol. 2023a;98:1465-1487.
  2. Tefferi A, Vannucchi AM, Barbui T. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. Am J Hematol. 2024;99:697-718.
  3. Pasquer H, Kiladjian JJ, Benajiba L. Current myeloproliferative neoplasm scoring systems for clinical practice. Blood. 2025;145:257-276.
  4. Rungjirajittranon T, Owattanapanich W, Ungprasert P, et al. A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms. BMC Cancer. 2019;19:184.
  5. Hultcrantz M, Björkholm M, Dickman PW, et al. Risk for arterial and venous thrombosis in patients with myeloproliferative neoplasms: a population-based cohort study. Ann Intern Med. 2018;168:317-325.
  6. 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.
  7. Stein BL, Martin K. From Budd-Chiari syndrome to acquired von Willebrand syndrome: thrombosis and bleeding complications in the myeloproliferative neoplasms. Blood. 2019;134:1902-1911.
  8. Kelliher S, Falanga A. Thrombosis in myeloproliferative neoplasms: a clinical and pathophysiological perspective. Thrombosis Update. 2021:5:100081.
  9. 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. 2015a;5:e369.
  10. Barbui T, Finazzi G, Carobbio A, et al. Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis). Blood. 2012;120:5128-5252.
  11. Haider M, Gangat N, Lasho T, et al. Validation of the revised International Prognostic Score of Thrombosis for Essential Thrombocythemia (IPSET-thrombosis) in 585 Mayo Clinic patients. Am J Hematol. 2016;91:390-394.
  12. Tefferi A, Loscocco GG, Farrukh F, et al. A globally applicable "triple A" risk model for essential thrombocythemia based on age, absolute neutrophil count, and absolute lymphocyte count. Am J Hematol. 2023b;98:1829-1837.
  13. Sonmez O, Oyur E, Yonal-Hindilerden I, et al. A practical tool for predicting outcomes in essential thrombocythemia: triple A risk model and beyond. Blood Adv. 2025;[Epub ahead of print].
  14. 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.
  15. National Comprehensive Cancer Network. Clinical practice guidelines in oncology: myeloproliferative neoplasms. v2.2025. Accessed January 26, 2026.
  16. 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.
  17. 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.
  18. Barbui T, Masciulli A, Marfisi MR, et al. White blood cell counts and thrombosis in polycythemia vera: a subanalysis of the CYTO-PV study. Blood. 2015b;126:560-561.
  19. Antithrombotic Trialists' (ATT) Collaboration, Baigent C, Blackwell L, et al. Aspirin in the primary and secondary prevention of vascular disease: collaborative meta-analysis of individual participant data from randomised trials. Lancet. 2009;373:1849-1860.
  20. Yusuf S, Bosch J, Dagenais G, et al. Cholesterol lowering in intermediate-risk persons without cardiovascular disease. N Engl J Med. 2016;374:2021-2031.
  21. Risk and Prevention Study Collaborative Group, Roncaglioni MC, Tombesi M, et al. n-3 fatty acids in patients with multiple cardiovascular risk factors. N Engl J Med. 2013;368:1800-1808.
  22. Barbui T, Carobbio A, Rumi E, et al. In contemporary patients with polycythemia vera, rates of thrombosis and risk factors delineate a new clinical epidemiology. Blood. 2014;124:3021-3023.
  23. Barbui T, Tefferi A, Vannucchi AM, et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia. 2018;32:1057-1069.
  24. Brown R, Jasiakiewicz J, Greer V, et al. Association between JAK2V617F variable allele frequency and risk of thrombotic events in patients with myeloproliferative neoplasms. Ir J Med Sci. 2024;193:2883-2888.
  25. Marnell CS, Bick A, Natarajan P. Clonal hematopoiesis of indeterminate potential (CHIP): Linking somatic mutations, hematopoiesis, chronic inflammation and cardiovascular disease. J Mol Cell Cardiol. 2021;161:98-105.
  26. Pasquer H, Daltro de Oliveira R, Vasseur L, et al. Distinct clinico-molecular arterial and venous thrombosis scores for myeloproliferative neoplasms risk stratification. Leukemia. 2024;38:326-339.
  27. Tefferi A, Guglielmelli P, Lasho TL, et al. Mutation-enhanced international prognostic systems for essential thrombocythaemia and polycythaemia vera. Br J Haematol. 2020;189:291-302.
  28. Grinfeld J, Nangalia J, Baxter EJ, et al. Classification and personalized prognosis in myeloproliferative neoplasms. N Engl J Med. 2018;379:1416-1430.