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
Review
. 2020 Jul 6;12(7):1810.
doi: 10.3390/cancers12071810.

Aetiology of Myeloproliferative Neoplasms

Mary Frances McMullin  1 , Lesley Ann Anderson  2
Affiliations
Review

Aetiology of Myeloproliferative Neoplasms

Mary Frances McMullin et al. Cancers (Basel). .

Abstract

Myeloproliferative neoplasms (MPNs) have estimated annual incidence rates for polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis of 0.84, 1.03, and 0.47 per 100,000. Prevalence is much higher, particularly for PV and ET, as mortality rates are relatively low. Patients are often concerned about why they developed an MPN and epidemiological studies enable the identification of potential causative factors. Previous work in small heterogeneous studies has identified a variety of risk factors associated with MPNs including family history of MPN, autoimmune conditions, some occupational exposures, and blood donation. At a population level, germline predisposition factors in various populations have been associated with MPNs. The pilot MOSAICC (Myeloproliferative Neoplasm: An In-depth Case-Control) study is one of the largest epidemiological studies in MPN ever carried out to date. It demonstrated the most effective methods for carrying out a significant epidemiological study in this patient group including the best way of recruiting controls, as well as how to evaluate occupational and lifestyle exposures, evaluate symptoms, and collect biological samples. Significant results linked to MPNs in the pilot study of 106 patients included smoking, obesity, and childhood socioeconomic status. The methodology is now in place for a much larger ongoing MOSAICC study which should provide further insight into the potential causes of MPNs.

Keywords: epidemiology; essential thrombocythemia; lifestyle factors; myeloproliferative neoplasms; polycythemia vera; smoking.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

References

    1. Arber D.A., Orazi A., Hasserjian R., Thiele J., Borowitz M.J., Le Beau M.M., Bloomfield C.D., Cazzola M., Vardiman J.W. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukaemia. Blood. 2016;127:2391–2405. doi: 10.1182/blood-2016-03-643544. - DOI - PubMed
    1. James C., Ugo V., Casadevall N., Constantinescu S.N., Vainchenker W. A JAK2 mutation in myeloproliferative disorders: Pathogenesis and therapeutic and scientific prospects. Trends. Mol. Med. 2005;11:546–554. doi: 10.1016/j.molmed.2005.10.003. - DOI - PubMed
    1. Pikman Y., Lee B.H., Mercher T., McDowell E., Ebert B.L., Gozo M., Cuker A., Wernig G., Moore S., Galinsky I., et al. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS Med. 2006;3:e270. doi: 10.1371/journal.pmed.0030270. - DOI - PMC - PubMed
    1. Nangalia J., Massie C.E., Baxter E.J., Nice F.L., Gundem G., Wedge D.C., Avezov E., Li J., Kollmann K., Kent D.G., et al. Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2. N. Engl. J. Med. 2013;369:2391–2405. doi: 10.1056/NEJMoa1312542. - DOI - PMC - PubMed
    1. Hultcrantz M., Kristinsson S.Y., Andersson T.M., Landgren O., Eloranta S., Derolf A.R., Dickman P.W., Björkholm M. Patterns of survival among patients with myeloproliferative neoplasms diagnosed in Sweden from 1973 to 2008: A population-based study. J. Clin. Oncol. 2012;30:2995–3001. doi: 10.1200/JCO.2012.42.1925. - DOI - PMC - PubMed

LinkOut - more resources