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The symptom burden, complications and prognosis of polycythaemia vera

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

Post-module multiple-choice questions

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Answer: According to the BSH diagnostic criteria, evidence of erythrocytosis – either haematocrit of >52% in men and >48% in women or a raised red cell mass >25% above predicted – plus the presence of a JAK2 mutation are required for a diagnosis of JAK2-mutation positive PV. Subnormal serum erythropoietin is a minor criterion in the diagnosis of the rare JAK2-mutation negative PV, along with other criteria including raised red cell mass, no cause of secondary erythrocytosis, relevant bone marrow histology and others.2

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Answer: Advanced age (≥65 years) is highly predictive of reduced survival in patients with PV.3,4 Haematocrit >45%, leukocytosis at diagnosis and venous thrombosis are also all associated with lower overall survival.3-5

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Answer: Increase in spleen size can cause symptoms such as abdominal pain and early satiety.6,7 Increase in circulating cytokines can cause inflammation-related symptoms such as dizziness, erythromelalgia and headache.7,8 Increased production of red blood cells can cause hyperviscosity-related symptoms such as fatigue, pruritus, night sweats and fever, as well as increase risk of thrombotic events.7,9

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Answer: In a study of 3,001 patients with PV, a 13.1-fold risk of venous thrombosis and a 2.7-fold risk of arterial thrombosis was observed 3 months after diagnosis, compared with a population of matched controls. The risk of thrombosis decreases with time.10

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BSH: British Society for Haematology; JAK2: Janus kinase-2

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. 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.
  4. 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.
  5. Marchioli R, Finazzi G et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med 2013;368(1):22-33.
  6. Tremblay D, Schwartz M et al. Modern management of splenomegaly in patients with myelofibrosis. Ann Hematol 2020;99(7):1441-1451.
  7. Vannucchi A M, Kiladjian J J et al. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med 2015;372(5):426-435./li>
  8. Cuthbert D, Stein B L. Polycythemia vera-associated complications: pathogenesis, clinical manifestations, and effects on outcomes. J Blood Med 2019;10:359-371.
  9. Kwaan H C, Wang J. Hyperviscosity in polycythemia vera and other red cell abnormalities. Semin Thromb Hemost 2003;29(5):451-458.
  10. Hultcrantz M, Bjorkholm M et al. Risk for arterial and venous thrombosis in patients with myeloproliferative neoplasms. Ann Intern Med 2018;169(4):268./li>