Polycythemia Vera


Polycythemia vera (PV) is a trilineage, Philadelphia chromosome–negative myeloproliferative neoplasm (MPN) characterized by chronic, unregulated proliferation of erythrocytes and leukocytes and/or platelets.1,2

Erythrocytosis (elevated total red cell mass) is the most prominent clinical expression of PV.2

Overactive JAK signaling plays a key role in the pathophysiology of PV.3,4 The JAK2 mutation V617F (or JAK2V617F) is a diagnostic feature of PV and is present in >95% of cases.2

PV affects about 100,000 people in the United States and occurs mostly in older people, with a median age of diagnosis of approximately 60 years.5-7 PV may be slightly more prevalent in men.6

Maintaining hematocrit (Hct) levels of <45% and controlling the clinical signs and symptoms of PV, including splenomegaly, are important clinical considerations in PV management.5,8 Clinical studies have shown, however, that more than 1 in 4 patients with PV had Hct levels ≥45% even with standard treatment that includes phlebotomy and hydroxyurea.9-11 In these patients, and those who continued to experience clinical signs and symptoms such as fatigue, pruritus, night sweats or splenomegaly, PV remained uncontrolled.


PV Can Affect All 3 Hematopoietic Cell Lines2
chart shows the origin of cell lines (common hematopoietic stem cell) where the <i>JAK2</i>V617F mutation happens and then the  breakout of the 3 different cell lines called granulocyte-monocyte progenitors, erythroid progenitors and megakaryocytic progenitors
JAK = Janus-associated kinase

1. Scherber RM, Geyer HL, Dueck AC, et al. Leuk Lymphoma. 2017;58(6):1481-1487. 2. Spivak JL. Ann Intern Med. 2010;152:300-306. 3. Rampal R, Al-Shahrour F, Abdel-Wahab O. Blood. 2014;123(22):e123-133. 4. Keohane C, Radia D, Harrison C. Biologics. 2013;7:189-198. 5. Passamonti F. Blood. 2012;120:275-284. 6. Stuart BJ, Viera AJ. Am Fam Physician. 2004;69:2139-2144. 7. Data on file. Incyte Corporation. Wilmington, DE. 8. Finazzi G, Barbui T. Blood. 2007;109:5104-5111. 9. Marchioli R, Finazzi G, Specchia G, et al; for the CYTO-PV Collaborative Group. N Engl J Med. 2013;368:22-33. 10. Marchioli R, Finazzi G, Landolfi R, et al. J Clin Oncol. 2005;23:2224-2232. 11. Di Nisio M, Barbui T, Di Gennaro LD, et al. Br J Haematol. 2006;136:249-259.

The potential impact of a target hematocrit level of <45% on major thrombosis or cardiac death.
By: MPN Connect
An animated video highlighting PV pathophysiology and mechanisms of disease.