Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Apr;287(4):448-454.
doi: 10.1111/joim.13019. Epub 2020 Jan 11.

Incidence of myeloproliferative neoplasms - trends by subgroup and age in a population-based study in Sweden

M Hultcrantz  1   2 , A Ravn Landtblom  3 , B Andréasson  4 , J Samuelsson  5 , P W Dickman  6 , S Y Kristinsson  7   8 , M Björkholm  1 , T M-L Andersson  6
Affiliations

Incidence of myeloproliferative neoplasms - trends by subgroup and age in a population-based study in Sweden

M Hultcrantz et al. J Intern Med. 2020 Apr.

Abstract

Background: The reported incidence of Philadelphia-negative myeloproliferative neoplasms (MPNs) differs substantially between previous reports, likely due to true regional differences in incidence and/or variations in the quality and coverage of the cancer registers.

Objective: We therefore assessed MPN incidence in Sweden during recent years using prospectively collected information captured in Swedish health registers.

Methods: Patients with MPNs were identified through the Swedish Cancer Register and Swedish Blood Cancer Register between 2000 and 2014. Information on the Swedish population was obtained from the Human Mortality Database. Crude and age-standardized incidence rates of MPNs with 95% confidence intervals (CIs) were calculated.

Results: A total of 6281 MPN cases were reported to the Swedish Cancer Register and Swedish Blood Cancer Register during 2000-2014. The age-standardized, to the Swedish population in 2000, incidence for all MPNs was 4.45 (95% confidence interval [CI] 4.34-4.56)/100 000 person-years. The age-standardized incidence for polycythemia vera was 1.48 (1.42-1.54), for essential thrombocythemia 1.60 (1.53-1.66) and for primary myelofibrosis 0.52 (0.48-0.56)/100 000 person-years, respectively. The incidence rate of MPNs was substantially higher in the older compared to the younger age groups. The incidence increased during the study period, likely to do better reporting and increasing age of the general population.

Conclusion: The reported MPN incidences in our study, which were in the higher interval of previously published studies, are likely more accurate compared to previous reports due to the population-based setting and high level of coverage in the Swedish Cancer and Blood Cancer Registers.

Keywords: essential thrombocythemia; incidence; myeloproliferative neoplasms; polycythemia vera; primary myelofibrosis.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest Statement:

The authors report no relevant conflicts of interest. TM Andersson is involved in an ongoing public-private real world evidence collaboration between Karolinska Institutet and Janssen Pharmaceuticals. However, the current project is not related to this collaboration.

Figures

Figure.
Figure.
Age-standardizeda incidence rate per 100,000 person-years of MPNs overall and MPN subtypes in Sweden between 2000 and 2014 a Swedish population in 2000 MPN= myeloproliferative neoplasm, PV= polycythemia vera, ET=essential thrombocythemia, PMF= primary myelofibrosis, MPN-U =myeloproliferative neoplasm unclassified
See this image and copyright information in PMC

References

    1. James C, Ugo V, Le Couedic JP, et al. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. Nature 2005; 434: 1144–8. - PubMed
    1. Baxter EJ, Scott LM, Campbell PJ, et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet 2005; 365: 1054–61. - PubMed
    1. Kralovics R, Passamonti F, Buser AS, et al. A gain-of-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med 2005; 352: 1779–90. - PubMed
    1. Levine RL, Wadleigh M, Cools J, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell 2005; 7: 387–97. - PubMed
    1. Klampfl T, Gisslinger H, Harutyunyan AS, et al. Somatic mutations of calreticulin in myeloproliferative neoplasms. N Engl J Med 2013; 369: 2379–90. - PubMed

Publication types