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The symptom burden, complications and prognosis of polycythaemia vera

Complications and progression

Polycythaemia vera (PV) increases the risk of thrombosis, associated complications and mortality1-3

Explore the diagram to learn more

*10-year risk for secondary cancers determined from 1,060 patients with an MPN including 553 with PV.5

How do I recognise disease progression?1

Clinical indicators:

  • development of myelofibrosis
  • elevated white blood cell count
  • presence of blasts in peripheral blood or bone marrow
  • reduced red blood cell count
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Symptomatic indicators:

  • occurrence of thromboembolic events; major bleeding
  • intensification of constitutional symptoms (including pruritis, night sweats, fever, weight loss and fatigue)

Thrombosis risk is higher in patients with PV than the average population2,13

Anya

As a person living with PV, Anya has a significantly higher risk of thrombosis than others her age13

A retrospective, cohort-based study in Sweden compared 9,429 patients with an MPN, (including 3,001 with PV) against 35,830 control subjects matched for ages, sex and period of diagnosis.† The study found that 3 months after PV diagnosis there was:13

  • a 2.7-fold (95% CI: 2.2-3.3) higher risk of arterial thrombosis
  • a 13.1-fold (95% CI: 8.7-19.6) higher risk of venous thrombosis

At 5 years post-diagnosis, the risk was 1.5x and 3.6x for arterial and venous thrombosis, respectively.13 This decrease in risk is likely due to the cytoreductive and thromboprophylactic effects of treatment.2,13

†Information on non-fatal and fatal arterial and venous thrombotic events was collated from the relevant national registers (1987-2009).

Thrombosis in PV is caused by elevated blood cell production increasing blood viscosity, reducing blood return through the veins and increasing platelet adhesion. Thrombotic events are responsible for nearly half of all deaths in patients with PV.2

Controlling haematocrit at <45% has been associated with reduced rates of cardiovascular death and major thrombotic events.2

Classifying risk in PV

Due to the significant effects of thrombosis on mortality and morbidity, risk in patients with PV can be classified according to thrombosis risk:14

Risk stratification
High risk Advanced age (≥65 years) and/or history of PV‑related thrombosis
Some patients classified as ‘low risk’ may be considered at higher risk in presence of: CV risk factors, elevated WBC count, thrombocytosis and/or elevated haematocrit (not controlled by phlebotomy)
Low risk Younger age (<65 years) and no history of PV‑related thrombosis

In clinical practice, over 50% of patients‡ with an MPN present with CV risk factors.15,16

A CV risk assessment should be considered for patients with PV every 5 years, or more often where risks are close to thresholds mandating treatment.17 All patients should be managed aggressively for their CV risk factors.18

‡Data collected by the Clinical Practice Research Datalink on 2,477 patients with an MPN.15

Continue to re-test your knowledge 

CV: cardiovascular; MPN: myeloproliferative neoplasm; WBC: white blood cell

JAKAVI® (ruxolitinib) is indicated for adult patients with PV who are resistant to or intolerant of hydroxyurea (also referred to as hydroxycarbamide in the UK).19

Adverse events should be reported. Reporting forms and information can be found at www.mhra.gov.uk/yellowcard. Adverse events should also be reported to Novartis online through the pharmacovigilance intake (PVI) tool at www.novartis.com/report or alternatively email medinfo.uk@novartis.com or call 01276 698370.

References
  1. Baumeister J, Chatain N et al. Progression of myeloproliferative neoplasms (MPN): diagnostic and therapeutic perspectives. Cells 2021;10(12)
  2. Griesshammer M, Kiladjian J J, Besses C. Thromboembolic events in polycythemia vera. Ann Hematol 2019;98(5):1071-1082
  3. Tefferi A, Rumi E et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia 2013;27(9):1874-1881
  4. Raedler L A. Diagnosis and management of polycythemia vera: proceedings from a multidisciplinary roundtable. Am Health Drug Benefits 2014;7(suppl 3):S36-47
  5. Zhang Y, Han Y et al. Incidence and risk factors for second malignancies among patients with myeloproliferative neoplasms. Cancer Med 2023;00:1-11
  6. Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management. Am J Hematol 2015;90(2):162-173
  7. Tefferi A, Vannucchi A M, Barbui T. Polycythemia vera: historical oversights, diagnostic details, and therapeutic views. Leukemia 2021;35(12):3339-3351
  8. Raedler L A. Diagnosis and management of polycythemia vera: proceedings from a multidisciplinary roundtable. Am Health Drug Benefits 2014;7(7 Suppl 3):S36-47
  9. Emanuel R M, Dueck A C et al. Myeloproliferative neoplasm (MPN) symptom assessment form total symptom score: prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs. J Clin Oncol 2012;30(33):4098-4103
  10. Passamonti F, Rumi E et al. A dynamic prognostic model to predict survival in post-polycythemia vera myelofibrosis. Blood 2008;111(7):3383-3387
  11. Passamonti F, Rumi E et al. A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications. Leukemia 2010;24(9):1574-1579
  12. Chihara D, Kantarjian H et al. Survival outcome of patients with acute myeloid leukemia transformed from myeloproliferative neoplasms. Blood 2016;128(22):1940
  13. Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management. Am J Hematol 2020;95(12):1599-1613
  14. Hultcrantz M, Bjorkholm M et al. Risk for arterial and venous thrombosis in patients with myeloproliferative neoplasms. Ann Intern Med 2018;169(4):268
  15. McMullin M F, Harrison C N et al. A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline. Br J Haematol 2019;184(2):176-191
  16. Chen F, Rabe A et al. An epidemiological study of the cardiovascular health and thrombotic risk profiles of patients with myeloproliferative neoplasms in primary care across the United Kingdom. Presented at European Hematology Association Annual Congress, virtual, 9-17 June 2021; EP1090
  17. Jentsch-Ullrich K, Eberhardt J et al. Characteristics and treatment of polycythemia vera patients in clinical practice: a multicenter chart review on 1476 individuals in Germany. J Cancer Res Clin Oncol 2016;142(9):2041-2049
  18. Visseren F L J, Mach F et al. 2021 ESC Guidelines on cardiovascular disease prevention in clinical practice. Eur Heart J 2021;42(34):3227-3337
  19. Barbui T, Tefferi A et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia 2018;32(5):1057-1069
  20. JAKAVI® Summary of Product Characteristics. Available at: www.medicines.org.uk (for GB) or www.emcmedicines.com (for NI). Accessed November 2024