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: Ifeoma Odukwe, M.D. [2] Mohamad Alkateb, MBBCh [3] Shyam Patel [4]

Overview

The incidence of polycythemia vera is approximately 1.9 per 100,000 individuals per year in the US. 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.

Epidemiology and Demographics

Incidence

  • The incidence of polycythemia vera is about 1.9 per 100,000 individuals per year in the US. The incidence rate for men is 2.8 per 100,000 person-years, and is 1.3 per 100,000 person years for women.[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]

Case-fatality rate/Mortality rate

  • The 15-year survival of patients with polycythemia vera is 65%.[4]
  • The mortality of polycythemia vera patients compared with the general population is 1.6-fold higher.

Age

  • The median age at diagnosis is 61 years but has been diagnosed in all age groups. The incidence increases with age.[5]
  • Younger patients have comparable rates of vascular complications compared to older patients. However, splanchnic vein thrombosis occur more frequently in younger patients.[6]
  • 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.[5]

Race

  • In ashkenazi jews, there is a high incidence of polycythemia vera.[7]

Gender

  • Males are more commonly affected with polycythemia vera than females. The male to female ratio is approximately 1.2 to 1.[5]

References

  1. Anía BJ, Suman VJ, Sobell JL, Codd MB, Silverstein MN, Melton LJ (1994). "Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989". Am J Hematol. 47 (2): 89–93. PMID 8092146.
  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. Passamonti F, Rumi E, Pungolino E, Malabarba L, Bertazzoni P, Valentini M; et al. (2004). "Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia". Am J Med. 117 (10): 755–61. doi:10.1016/j.amjmed.2004.06.032. PMID 15541325.
  5. 5.0 5.1 5.2 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.
  6. 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.
  7. Vannucchi AM (2017). "From leeches to personalized medicine: evolving concepts in the management of polycythemia vera". Haematologica. 102 (1): 18–29. doi:10.3324/haematol.2015.129155. PMC 5210229. PMID 27884974.

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