The symptom burden, complications and prognosis of polycythaemia vera
Post-module multiple-choice questions
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Answer: Phlebotomy and low-dose aspirin are recommended for all patients with PV with the aim of maintaining haematocrit <45%. Cytoreduction is recommended in high-risk patients, for example those >60 years old or with a history of a thrombotic event, and can be considered in cases of splenomegaly, severe symptoms, thrombocytosis and leukocytosis. HU or recombinant interferon alfa can be used as first-line cytoreductive therapy; caution should be used for when considering HU for younger patients.1
Please note that aspirin is not licensed for the treatment of PV and neither are all recombinant interferon alfa therapies. Please refer to individual Summaries of Product Characteristics before prescribing.
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Answer: The modified ELN criteria for HU intolerance/ resistance include the need for unacceptably frequent phlebotomy after at least 3 months of HU therapy (≥2 g/day of HU or maximum tolerated dose). Other criteria that may be indicative of intolerance or resistance include: uncontrolled myeloproliferation; low absolute neutrophil count, platelet count or haemoglobin; failure to reduce massive splenomegaly or control symptoms; disease-related thrombosis or haemorrhage; presence of HU-related toxicity eg leg ulcer, mucocutaneous issues, gastrointestinal symptoms, pneumonitis or fever.2
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Answer: In a study of 261 Spanish patients with PV receiving HU treatment, the median survival from diagnosis was 19 years, from start of HU therapy was 18 years and from onset of HU resistance was 1.2 years.3
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Answer: Sustained leukocytosis despite HU treatment was found to reduce overall survival (HR: 2.7, 95% CI: 1.3-5.4, p=0.007, n=261) and increase the chance of transformation to myelofibrosis or acute leukaemia (HR: 3.2, 95% CI: 1.5-7.1, p=0.004, n=261).3 Increased risk of mucocutaneous lesions can be associated with HU use.4
BSH: British Society for Haematology; JAK2: Janus kinase-2
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).5
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
- Barbui T, Tefferi A et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia 2018;32(5):1057-1069
- McMullin M F, Wilkins B S, Harrison C N. Management of polycythaemia vera: a critical review of current data. Br J Haematol 2016;172(3):337-349
- 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
- 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
- JAKAVI® Summary of Product Characteristics. Available at: www.medicines.org.uk (for GB) or www.emcmedicines.com (for NI). Accessed November 2024