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The treatment landscape
for polycythaemia vera

JAKAVI® (ruxolitinib) is indicated for adult patients with polycythaemia vera who are resistant to or intolerant of hydroxyurea (also referred to as hydroxycarbamide in the UK).1

Post-module multiple-choice questions

  • Not all forms of interferon therapy are licensed for use in PV
  • Pipobroman is not licensed for use in PV in the UK
  • 32P is not licensed for use in PV in the UK
  • Please refer to the relevant SmPC before prescribing

Hydroxyurea is also referred to in the UK as hydroxycarbamide.

Correct answers are highlighted.

Answer: The BSH recommends hydroxyurea or interferon (preferably pegylated interferon) as first-line options for high‑risk PV.1

Correct answers are highlighted.

Answer: Hydroxyurea can reduce HCT concentration and platelet count,3 and has been shown to reduce thrombotic events versus phlebotomy alone (9.8% versus 32.8%, respectively; p=0.009).4 Hydroxyurea is generally well tolerated;2 however, patients may become resistant or intolerant to it, indicating the need for a change in therapy.5 Hydroxyurea is recommended by the BSH as a first‑line treatment for high-risk patients with PV, or as second‑line therapy following first‑line interferon.2

Hydroxyurea is also referred to in the UK as hydroxycarbamide.

Correct answer is highlighted.

Answer: Patients with PV can suffer from aquagenic pruritus, a debilitating condition characterised by strong itching, stinging, tingling, or burning sensations upon contact with water.6 Antihistamines can be used but are largely ineffective.7 Topical agents such as cooling cream, lidocaine cream, capsaicin or corticosteroids may be helpful, and patients can try to reduce discomfort by lubricating the skin and minimising bathing.8

Correct answer is highlighted.

Answer: All eligible patients with PV should be managed with phlebotomy; targeting HCT <45% reduces thrombosis risk and is recommended by the ELN and BSH guidelines.2,9 However, phlebotomy is generally unsuitable for long-term treatment; a ≥15‑year follow-up analysis found it increases the risk of early progression to myelofibrosis with myeloid splenomegaly.10 Iron deficiency can also develop in patients who receive phlebotomies over a long period of time.3

Correct answer is highlighted.

Answer: The BSH recommends considering pegylated interferon as second line in patients who have had non-pegylated interferon first line and could not tolerate it.2

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Treatment for patients with PV should be considered on an individual basis based on their clinical condition, preferences, and medical needs. Always consult the guidelines and product Summaries of Product Characteristics before prescribing a treatment.

32P: phosphorus-32; BSH: British Society for Haematology; ELN: European LeukemiaNet; HCT: haematocrit; PV: polycythaemia vera

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. 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.
  3. Leukemia and Lymphoma Society. Polycythemia vera facts. Available at: lls.org. [Accessed March 2025].
  4. Griesshammer M, Kiladjian J J, Besses C. Thromboembolic events in polycythemia vera. Ann Hematol 2019;98(5):1071-1082.
  5. Alvarez-Larran A, Pereira A et al. Assessment and prognostic value of the European LeukemiaNet criteria for clinicohematologic response, resistance, and intolerance to hydroxyurea in polycythemia vera. Blood 2012;119(6):1363-1369.
  6. Siegel F P, Tauscher J, Petrides P E. Aquagenic pruritus in polycythemia vera: characteristics and influence on quality of life in 441 patients. Am J Hematol 2013;88(8):665-669.
  7. Passamonti F. How I treat polycythemia vera. Blood 2012;120(2):275-284.
  8. Seccareccia D, Gebara N. Pruritus in palliative care: getting up to scratch. Can Fam Physician 2011;57(9):1010-1013, e1316-1019.
  9. Barbui T, Tefferi A et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia 2018;32(5):1057-1069.
  10. Najean Y, Dresch C, Rain J D. The very-long-term course of polycythaemia: a complement to the previously published data of the Polycythaemia Vera Study Group. Br J Haematol 1994;86(1):233-235.