In the US, incidence of polycythemia vera (PV) is reported to be 1.57 per 100,000 person-years (data from 2002 to 2016).[11]Verstovsek S, Yu J, Scherber RM, et al. Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States. Leuk Lymphoma. 2022 Mar;63(3):694-702.
https://www.tandfonline.com/doi/full/10.1080/10428194.2021.1992756
http://www.ncbi.nlm.nih.gov/pubmed/34689695?tool=bestpractice.com
In the UK, annual incidence of PV is 1.8 per 100,000, with an estimated 1140 people newly diagnosed each year (data from 2010 to 2019).[20]Haematological Malignancy Research Network (HMRN). Cancer factsheets: polycythaemia vera. May 2024 [internet publication].
https://hmrn.org/factsheets#polycythaemia_vera
PV occurs more commonly in males (male to female rate ratio approximately 1.6 in the US and 1.2 in the UK).[11]Verstovsek S, Yu J, Scherber RM, et al. Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States. Leuk Lymphoma. 2022 Mar;63(3):694-702.
https://www.tandfonline.com/doi/full/10.1080/10428194.2021.1992756
http://www.ncbi.nlm.nih.gov/pubmed/34689695?tool=bestpractice.com
[20]Haematological Malignancy Research Network (HMRN). Cancer factsheets: polycythaemia vera. May 2024 [internet publication].
https://hmrn.org/factsheets#polycythaemia_vera
Incidence in the US is reported to be highest among white people.[11]Verstovsek S, Yu J, Scherber RM, et al. Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States. Leuk Lymphoma. 2022 Mar;63(3):694-702.
https://www.tandfonline.com/doi/full/10.1080/10428194.2021.1992756
http://www.ncbi.nlm.nih.gov/pubmed/34689695?tool=bestpractice.com
Increased incidence and prevalence of PV has been reported in Ashkenazi Jewish communities.[21]Modan B, Kallner H, Zemer D, et al. A note on the increased risk of polycythemia vera in Jews. Blood. 1971 Feb;37(2):172-6.
http://bloodjournal.hematologylibrary.org/cgi/reprint/37/2/172
http://www.ncbi.nlm.nih.gov/pubmed/5549194?tool=bestpractice.com
[22]Chaiter Y, Brenner B, Aghai E, et al. High incidence of myeloproliferative disorders in Ashkenazi Jews in northern Israel. Leuk Lymphoma. 1992 Jun;7(3):251-5.
http://www.ncbi.nlm.nih.gov/pubmed/1477653?tool=bestpractice.com
[23]Le M, Ghazawi FM, Rahme E, et al. Identification of significant geographic clustering of polycythemia vera cases in Montreal, Canada. Cancer. 2019 Nov 15;125(22):3953-9.
https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.32417
http://www.ncbi.nlm.nih.gov/pubmed/31381139?tool=bestpractice.com
PV more commonly affects middle-aged and older people (approximately 63% of patients in the US are age ≥60 years at diagnosis), but it can occur at any age.[11]Verstovsek S, Yu J, Scherber RM, et al. Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States. Leuk Lymphoma. 2022 Mar;63(3):694-702.
https://www.tandfonline.com/doi/full/10.1080/10428194.2021.1992756
http://www.ncbi.nlm.nih.gov/pubmed/34689695?tool=bestpractice.com
Median age at diagnosis is 65 years in the US and 71 years in the UK.[11]Verstovsek S, Yu J, Scherber RM, et al. Changes in the incidence and overall survival of patients with myeloproliferative neoplasms between 2002 and 2016 in the United States. Leuk Lymphoma. 2022 Mar;63(3):694-702.
https://www.tandfonline.com/doi/full/10.1080/10428194.2021.1992756
http://www.ncbi.nlm.nih.gov/pubmed/34689695?tool=bestpractice.com
[20]Haematological Malignancy Research Network (HMRN). Cancer factsheets: polycythaemia vera. May 2024 [internet publication].
https://hmrn.org/factsheets#polycythaemia_vera
Prevalence of PV has been reported to be between 22 and 57 per 100,000 in the US.[9]Johansson P. Epidemiology of the myeloproliferative disorders polycythemia vera and essential thrombocythemia. Semin Thromb Hemost. 2006 Apr;32(3):171-3.
http://www.ncbi.nlm.nih.gov/pubmed/16673273?tool=bestpractice.com
[24]Ma X, Vanasse G, Cartmel B, et al. Prevalence of polycythemia vera and essential thrombocythemia. Am J Hematol. 2008 May;83(5):359-62.
http://www.ncbi.nlm.nih.gov/pubmed/18181200?tool=bestpractice.com
[25]Titmarsh GJ, Duncombe AS, McMullin MF, et al. How common are myeloproliferative neoplasms? A systematic review and meta-analysis. Am J Hematol. 2014 Jun;89(6):581-7.
https://onlinelibrary.wiley.com/doi/10.1002/ajh.23690
http://www.ncbi.nlm.nih.gov/pubmed/24971434?tool=bestpractice.com
[26]Mehta J, Wang H, Iqbal SU, et al. Epidemiology of myeloproliferative neoplasms in the United States. Leuk Lymphoma. 2014 Mar;55(3):595-600.
http://www.ncbi.nlm.nih.gov/pubmed/23768070?tool=bestpractice.com
Global data, outside of the US and Western Europe, are scant.
PV is extremely rare in children.[10]Cario H, McMullin MF, Pahl HL. Clinical and hematological presentation of children and adolescents with polycythemia vera. Ann Hematol. 2009 Aug;88(8):713-9.
http://www.ncbi.nlm.nih.gov/pubmed/19468728?tool=bestpractice.com
[27]Ishida H, Miyajima Y, Hyakuna N, et al. Clinical features of children with polycythemia vera, essential thrombocythemia, and primary myelofibrosis in Japan: a retrospective nationwide survey. EJHaem. 2020 Jul;1(1):86-93.
https://onlinelibrary.wiley.com/doi/10.1002/jha2.39
http://www.ncbi.nlm.nih.gov/pubmed/35847744?tool=bestpractice.com