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Timely management of polycythaemia vera: considerations and pitfalls

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).1

Introduction

Polycythaemia vera (PV) is a myeloproliferative disorder that causes overproduction of red blood cells and is characterised by high symptom burden, risk of thrombotic events and disease transformation, and shortened life expectancy.2,3

The recommended treatment for patients with PV at high risk of thrombotic events is cytoreductive therapy, such as hydroxyurea (HU).2,3 HU is an established and commonly used first-line cytoreductive agent, which can help control red blood cell volume (haematocrit), and can reduce risk of thrombosis versus phlebotomy alone.2,4,5 However, in some cases, intolerance or resistance to HU therapy can occur, in which case a change in therapy is warranted; limited alternative options are available.4

Learning objectives

  • Be aware of the different treatment options for patients with PV, including recommended first-line cytoreduction therapy
  • Be able to recognise intolerance or lack of response to HU therapy
  • Understand the risks associated with insufficient control of PV

Multiple-choice questions to test your current knowledge

These questions will help you assess your current knowledge of this topic before you begin to work on the module. Your answers will be marked but will not count towards your final score. You will be asked the same questions again at the end of the module and will be able to download a learning certificate for your records upon completion.

Low-dose aspirin and some forms of interpheron alpha are not licensed in the UK for treating PV. Please refer to the SmPC before prescribing.

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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. JAKAVI® Summary of Product Characteristics. Available at: medicines.org.uk.
  2. Tefferi A, Vannucchi A M, Barbui T. Polycythemia vera: historical oversights, diagnostic details, and therapeutic views. Leukemia 2021;35(12):3339-3351.
  3. Harrison C N, Koschmieder S et al. The impact of myeloproliferative neoplasms (MPNs) on patient quality of life and productivity: results from the international MPN Landmark survey. Ann Hematol 2017;96(10):1653-1665.
  4. Barbui T, Tefferi A et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia 2018;32(5):1057-1069.
  5. Alvarez-Larran A, Perez-Encinas M et al. Risk of thrombosis according to need of phlebotomies in patients with polycythemia vera treated with hydroxyurea. Haematologica 2017;102(1):103-109.