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A JAK2 mutated HSC
is the source of PV
in nearly all patients2

PV is a myeloproliferative neoplasm (MPN)—a rare blood cancer that begins with the acquisition of a disease-initiating mutation by a single HSC in the bone marrow.1,3

Get the facts on
PV epidemiology

Despite being rare, PV is the most common MPN in the United States3,4

As many as 160,000 people in the United States are living with PV4,5Approximately 50 cases occur per every 100,000 people

Median age at diagnosis is 61 years, but
nearly 30% of cases occur in patients younger than 60 years6,7Diagnosis typically involves blood and genetic testing as well as bone marrow biopsy8

PV is slightly more common in men
(54% vs 46%)7

Potential consequences include thrombosis, progression to myelofibrosis (MF), and/or transformation to acute myeloid leukemia (AML)3

Median overall survival for PV is
15 years9

In more than 95% of patients with PV, a JAK2 mutation drives pathogenesis.1,2,10 As the disease develops, the mutation confers a selective advantage that favors malignant clone expansion over normal cells.1 PV is also characterized by heterogeneity in disease severity, which explains the variability in patient outcomes.10,11 There is no reliable way to predict who may progress and when.

Expansion of the JAK2 malignant clone ultimately accounts for the short- and long-term consequences
of PV1,3,12

Learn more about the clinical consequences

The clinical consequences of PV

PV is characterized by excessive levels of circulating erythrocytes, as well as leukocytes and platelets, which may contribute to serious complications throughout the course of the disease3,11

Up to 5.5 thrombotic events occur per 100 patients each year13

PV is associated with cardiovascular
(CV) mortality In the ECLAP study, 1638 patients with PV
were enrolled14164 deaths were recorded45% of all deaths were attributed
to CV mortality

Progression to MF occurs in up to 21% of patients, typically 8 to 20 years
post diagnosis6,15,16Median overall survival of 48 months was demonstrated in patients with post-PV MF17

Leukemic transformation occurs in up to 10% of patients, typically >10 years post diagnosis6,15,16Transformation has been associated with
the acquisition of additional mutations
during PV progression18,19

ECLAP, European Collaboration on Low-Dose Aspirin in Polycythemia Vera.

Current management may not address the source of PV, which increases the likelihood of disease sequelae1,20-22

Learn more

Ideally, the management of PV would be focused on achieving a sustained impact against the expansion of the JAK2 malignant clone1,20-22

Although PV is characterized by heterogeneity in disease severity, >80% of patients follow a similar treatment journey11,23

Management of PV would ideally be focused on not only clinical parameters—hematocrit, thrombotic risk, and symptom burden—but also on the key molecular drivers of the disease, which may include high or increasing mutant JAK2 allelic burden* and the acquisition of additional mutations1,20-22

PV management should focus on providing a sustained impact
across the totality
of the disease

*Mutant JAK2 allelic burden refers to the proportion of the mutant alleles in relation to total JAK2 alleles (ie, JAK2 V617F/(JAK2 V617F + wild-type JAK2) × 100).24

Explore the Source of PV

Early intervention
at the source of PV―
the
JAK2 mutated stem cell—is fundamental to impact the short- and long-term sequelae of the disease before they develop.18,25

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References: 1. Mead AJ, Mullally A. Myeloproliferative neoplasm stem cells. Blood. 2017;129(12):1607-1616. 2. Tefferi A, Vanucchi AM, Barbui T. Polycythemia vera treatment algorithm 2018. Blood Cancer J. 2018;8(1):3. 3. Shaikh SS, Stein BL. Polycythemia vera: contemporary updates in diagnosis, prognosis, and treatment. Am J Hematol Oncol. 2017;13(9)23-31. 4. Mehta J, Wang H, Iqbal SU, Mesa R. Epidemiology of myeloproliferative neoplasms in the United States. Leuk Lymphoma. 2014;55(3):595-600. 5. United States Census Bureau. US and World Population Clock. https://www.census.gov/popclock. Accessed October 23, 2020. 6. Stein BL, Oh ST, Berenzon D, et al. Polycythemia vera: an appraisal of the biology and management 10 years after the discovery of JAK2 V617F. J Clin Oncol. 2015;33(33):3953-3960. 7. Mesa R, Boccia RV, Grunwald MR. Patient-
reported outcomes data from REVEAL at the time of enrollment (baseline): a prospective observational study of patients with polycythemia vera in the United States. Clin Lymphoma Myeloma Leuk. 2018;18(9):590-596. 8. Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management. Am J Hematol. 2019;94(1):133-143. 9. Szuber N, Mudireddy M, Nicolosi M, et al. 3023 Mayo Clinic patients with myeloproliferative neoplasms: risk-stratified comparison of survival and outcomes data among disease subgroups. Mayo Clin Proc. 2019;94(4):599-610. 10. Palumbo GA, Stella S, Pennisi MS, et al. The role of new technologies in myeloproliferative neoplasms. Front Oncol. 2019;9:321. 11. Raedler LA. Diagnosis and management of polycythemia vera: proceedings from a multidisciplinary roundtable. Am Health Drug Benefits. 2014;7(7 suppl 3):S36-S47. 12. Lussana F, Rambaldi A. Inflammation and myeloproliferative neoplasms. J Autoimmun. 2017;85:58-63. 13. Finazzi G. A prospective analysis of thrombotic events in the European collaboration study on low-dose aspirin in polycythemia (ECLAP). Pathol Biol (Paris). 2004;52(5):285-288. 14. Marchioli R, Finazzi G, Landolfi R, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. 2005;23(10):
2224-2232. 15. Cerquozzi S, Tefferi A. Blast transformation and fibrotic progression in polycythemia vera and essential thrombocythemia: a literature review of incidence and risk factors. Blood Cancer J. 2015;5(11):e366. 16. Tefferi A, Barbui T. Essential thrombocythemia and polycythemia vera: focus on clinical practice. Mayo Clin Proc. 2015;90(9):1283-1293. 17. Masarova L, Bose P, Daver N, et al. Patients with post-essential thrombocythemia and post-polycythemia vera differ from patients with primary myelofibrosis. Leuk Res.
2017;59:110-116. 18. Hasselbalch HC, Holmström MO. Perspectives on interferon-alpha in the treatment of polycythemia vera and related myeloproliferative neoplasms: minimal residual disease and cure? Semin Immunopathol. 2019;41(1):5-19. 19. Cuthbert D, Stein BL. Polycythemia vera–associated complications: pathogenesis, clinical manifestations, and effects on outcomes. J Blood Med. 2019;10:359-371. 20. Larsen TS, Pallisgaard N, de Stricker K, Møller MB, Hasselbalch HC. Limited efficacy of hydroxyurea in lowering of the JAK2 V617F allele burden. Hematology. 2009;14(1):11-15. 21. Zalcberg IR, Ayres-Silva J, de Azevedo AM, et al. Hydroxyurea dose impacts hematologic parameters in polycythemia vera and essential thrombocythemia but does not appreciably affect JAK2-V617F allele burden. Haematologica. 2011;96(3):e18-e20. 22. Austin RJ, Straube J, Bruedigam C. Distinct effects of ruxolitinib and interferon-alpha on murine JAK2V617F myeloproliferative neoplasm hematopoietic stem cell populations. Leukemia. 2020;34(4)1075-1089. 23. Data on file. PharmaEssentia USA Corp. Burlington, MA. 2020. 24. Tefferi A, Strand JJ, Lasho TL, et al. Bone marrow JAK2V617F allele burden and clinical correlates in polycythemia vera. Leukemia. 2007;21(9):
2074-2075. 25. Craver BM, Alaoui KE, Scherber RM, Fleischman AG. The critical role of inflammation in the pathogenesis and progression of myeloid malignancies. Cancers (Basel). 2018;10(4):104.