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The symptom burden, complications and prognosis of polycythaemia vera

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.1

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Answer: Advanced age (≥65 years) is highly predictive of reduced survival in patients with PV.2,3 Haematocrit >45%, leukocytosis at diagnosis and venous thrombosis are also all associated with lower overall survival.2-4

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Answer: Increase in spleen size can cause symptoms such as abdominal pain and early satiety.5,6 Increase in circulating cytokines can cause inflammation-related symptoms such as dizziness, erythromelalgia and headache.6,7 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.6,8

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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.9

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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).10

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. 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
  2. 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
  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. Marchioli R, Finazzi G et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med 2013;368(1):22-33
  5. Tremblay D, Schwartz M et al. Modern management of splenomegaly in patients with myelofibrosis. Ann Hematol 2020;99(7):1441-1451
  6. 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
  7. Cuthbert D, Stein B L. Polycythemia vera-associated complications: pathogenesis, clinical manifestations, and effects on outcomes. J Blood Med 2019;10:359-371
  8. Kwaan H C, Wang J. Hyperviscosity in polycythemia vera and other red cell abnormalities. Semin Thromb Hemost 2003;29(5):451-458
  9. Hultcrantz M, Bjorkholm M et al. Risk for arterial and venous thrombosis in patients with myeloproliferative neoplasms. Ann Intern Med 2018;169(4):268
  10. JAKAVI® Summary of Product Characteristics. Available at: www.medicines.org.uk (for GB) or www.emcmedicines.com (for NI). Accessed November 2024