Prescribing information (hosted externally)

The symptom burden, complications and prognosis of polycythaemia vera

A day in the life: the burden of polycythaemia vera

Polycythaemia vera (PV) has a high symptom burden, detrimentally impacting patients’ quality of life.1,2

I couldn’t even get my head off the pillow, let alone go up the stairs3

Anya

It’s not a scratchable itch. It consumes you. I haven’t had a full shower or bath for 9 years now because it’s triggered by water3

Real patient quotes

Up to 88%

of patients with PV experience fatigue1

More than 1 in 2

patients with PV experience pruritus1

Self-reported data from 538 patients with PV.1

  • 7 in 10 patients felt symptoms reduced their quality of life2
  • 3 in 5 patients reported symptom reduction as their primary goal of treatment2

Data from 223 patients with PV in the international MPN Landmark survey.2

Patients with PV may suffer a range of symptoms2

Abnormal JAK signalling in PV causes symptoms driven by:

  • increased production of red blood cells causing hyperviscosity4,5
  • increase in circulating inflammatory cytokines6
  • extramedullary haematopoiesis in the spleen causing spleen enlargement7

Explore the prevalence of different symptoms:

Cytokine-related symptoms ›
Hyperviscosity-related symptoms ›
Splenomegaly-related symptoms ›

Emotional impact of the PV symptom burden

Anya experiences severe aquagenic pruritus, fatigue, headache and difficulty concentrating. She has periods of depression and anxiety, and worries about her disease worsening.

Anya
  • The overall physical symptom burden of MPNs is associated with depression9
  • Aquagenic pruritus increases the likelihood of depression in patients with PV (24% vs 8% in patients without aquagenic pruritus; n=102; p <0.03)10

Emotional impact of MPN symptom burden on daily life:11

graph-1

Adapted from Harrison et al. 201911

Data from 286 patients with MPNs (163 with essential thrombocytopenia, 45 with myelofibrosis and 78 with PV) surveyed in the UK MPN Landmark Survey. Symptom severity was calculated by combining the number of symptoms with their severity for each patient, and patients allocated into quartiles (quartile 1: lowest symptom burden; quartile 4: highest symptom burden).11

Managing symptoms of PV may help to alleviate the emotional burden

Objective assessment of symptom burden

The MPN-symptom assessment form - total symptom score (MPN-SAF TSS) is a validated instrument intended to:1

  • quantitatively assess the burden of symptoms in patients with MPNs
  • monitor progression of disease
  • assess response to treatment
mpn assessment form

This MPN10 symptom tracker has been developed and funded by Novartis.

What are the risks and complications associated with PV? 

JAK: Janus kinase; MPN: myeloproliferative neoplasm

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).12

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. Emanuel R M, Dueck A C et al. Myeloproliferative neoplasm (MPN) symptom assessment form total symptom score: prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs. J Clin Oncol 2012;30(33):4098-4103
  2. 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
  3. Data on file, Novartis. Patient Quotes in PV from Advisory Board [MPN_PV_2020_001]. November 2020
  4. Kwaan H C, Wang J. Hyperviscosity in polycythemia vera and other red cell abnormalities. Semin Thromb Hemost 2003;29(5):451-458
  5. Vainchenker W, Kralovics R. Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms. Blood 2017;129(6):667-679
  6. Cuthbert D, Stein B L. Polycythemia vera-associated complications: pathogenesis, clinical manifestations, and effects on outcomes. J Blood Med 2019;10:359-371
  7. Tremblay D, Schwartz M et al. Modern management of splenomegaly in patients with myelofibrosis. Ann Hematol 2020;99(7):1441-1451
  8. 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
  9. McFarland D C, Shaffer K M et al. Associations of physical and psychologic symptom burden in patients with Philadelphia chromosome-negative myeloproliferative neoplasms. Psychosomatics 2018;59(5):472-480
  10. Lelonek E, Matusiak L et al. Burden of aquagenic pruritus in polycythaemia vera. Acta Derm Venereol 2018;98(2):185-190
  11. Harrison C, Mathias J et al. UK results from the myeloproliferative neoplasms (MPN) landmark survey on the symptom, emotional and economic burden of MPN. Br J Haematol 2019;186(3):e1-e4
  12. JAKAVI® Summary of Product Characteristics. Available at: www.medicines.org.uk (for GB) or www.emcmedicines.com (for NI). Accessed November 2024