Polycythemia vera epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]; Shyam Patel [3]

Overview

The incidence of polycythemia vera is approximately 0.7 to 2.6 per 100,000 individuals in the US.[1] The prevalence of polycythemia vera is 48 to 57 cases per 100,000 individuals in the United States. Males are more commonly affected than females. The life expectancy is variable but is typically more than 10 years for patients who have uncomplicated polycythemia vera and is significantly shorter in the case of post-PV myelofibrosis or post-PV acute myeloid leukemia.

Epidemiology and Demographics

Incidence

  • The incidence of polycythemia vera is approximately 0.7 to 2.6 per 100,000 individuals in the US.[1]

Prevalence

  • The prevalence of PV is estimated to be 44 to 57 cases per 100,000 persons, and approximately 148,000 persons are living with PV in the United States.[2][3]

Age

  • Polycythemia vera occurs in all age groups (including children), the incidence increases with age.
  • The median age at diagnosis is 61 years but has been diagnosed in all age groups (including children).[4]
  • Younger patients have comparable rates of vascular complications compared to older patients. However, splanchnic vein thrombosis occur more frequently in younger patients.[5]
  • Younger patients are more likely to develop unusual complications such as mesenteric venous thrombosis. The rate of transformation to acute leukemia is similar as that of older adults.[4]

Gender

  • Males are more commonly affected with polycythemia vera than females. However, about 75% of patients less than 40 years who are diagnosed are females.[4]

Life expectancy

  • The life expectancy after diagnosis is 13.5 years.[6] For patients who develop post-PV myelofibrosis or post-PV acute myeloid leukemia, the life expectancy is significantly shorter. Please see Natural History, Complications, and Prognosis section.

References

  1. 1.0 1.1 National Cancer Institute. Polycythemia vera 2015.http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd538d/
  2. Mehta, Jyotsna; Wang, Hongwei; Iqbal, Sheikh Usman; Mesa, Ruben (2013). "Epidemiology of myeloproliferative neoplasms in the United States". Leukemia & Lymphoma. 55 (3): 595–600. doi:10.3109/10428194.2013.813500. ISSN 1042-8194.
  3. Stein, Brady L.; Oh, Stephen T.; Berenzon, Dmitriy; Hobbs, Gabriela S.; Kremyanskaya, Marina; Rampal, Raajit K.; Abboud, Camille N.; Adler, Kenneth; Heaney, Mark L.; Jabbour, Elias J.; Komrokji, Rami S.; Moliterno, Alison R.; Ritchie, Ellen K.; Rice, Lawrence; Mascarenhas, John; Hoffman, Ronald (2015). "Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery ofJAK2 V617F". Journal of Clinical Oncology. 33 (33): 3953–3960. doi:10.1200/JCO.2015.61.6474. ISSN 0732-183X.
  4. 4.0 4.1 4.2 Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R (November 2015). "Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F". J. Clin. Oncol. 33 (33): 3953–60. doi:10.1200/JCO.2015.61.6474. PMC 4979103. PMID 26324368.
  5. Stein BL, Saraf S, Sobol U, Halpern A, Shammo J, Rondelli D, Michaelis L, Odenike O, Rademaker A, Zakarija A, McMahon B, Spivak JL, Moliterno AR (September 2013). "Age-related differences in disease characteristics and clinical outcomes in polycythemia vera". Leuk. Lymphoma. 54 (9): 1989–95. doi:10.3109/10428194.2012.759656. PMID 23245211.
  6. Verstovsek S, Komrokji RS (2015). "Novel and emerging therapies for the treatment of polycythemia vera". Expert Rev Hematol. 8 (1): 101–13. doi:10.1586/17474086.2015.972359. PMC 4934659. PMID 25353086.

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