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Review
. 2021 Apr;35(2):177-189.
doi: 10.1016/j.hoc.2020.11.005. Epub 2021 Jan 4.

Epidemiology of the Philadelphia Chromosome-Negative Classical Myeloproliferative Neoplasms

Affiliations
Review

Epidemiology of the Philadelphia Chromosome-Negative Classical Myeloproliferative Neoplasms

Rory M Shallis et al. Hematol Oncol Clin North Am. 2021 Apr.

Abstract

Polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) comprise the BCR-ABL-negative classical myeloproliferative neoplasms (MPNs). These clonal myeloid diseases are principally driven by well-described molecular events; however, factors leading to their acquisition are not well understood. Beyond increasing age, male sex, and race/ethnicity differences, few consistent risk factors for the MPNs are known. PV and ET have an incidence of 0.5 to 4.0 and 1.1 to 2.0 cases per 100,000 person-years, respectively, and predict similar survival. PMF, which has an incidence of about 0.3 to 2.0 cases per 100,000 person-years, is associated with the shortest survival of the MPNs.

Keywords: Epidemiology; Incidence; Myeloproliferative neoplasms; Prevalence; Survival.

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Conflict of interest statement

Disclosures/competing interests A.M. Zeidan received research funding (institutional) from Celgene, Acceleron, Abbvie, Otsuka, Pfizer, Medimmune/AstraZeneca, Boehringer Ingelheim, Trovagene, Incyte, Takeda, and ADC Therapeutics. A.M. Zeidan had a consultancy with and received honoraria from AbbVie, Otsuka, Pfizer, Celgene, Ariad, Incyte, Agios, Boehringer-Ingelheim, Novartis, Acceleron, Astellas, Daiichi Sankyo, Cardinal Health, Seattle Genetics, BeyondSpring, and Takeda. N.A. Podoltsev consulted for and received honoraria from Alexion, Pfizer, Agios Pharmaceuticals, Blueprint Medicines, Incyte, Novartis, Celgene, Bristol-Myers Squib, CTI BioPharma, and PharmaEssentia. N.A. Podoltsev received research funding (all to the institution) from Boehringer Ingelheim, Astellas Pharma, Daiichi-Sankyo, Sunesis Pharmaceuticals, Jazz Pharmaceuticals, Pfizer, Astex Pharmaceuticals, CTI biopharma, Celgene, Genentech, AI Therapeutics, Samus Therapeutics, Arog Pharmaceuticals, and Kartos Therapeutics. None of these relationships were related to the development of this article. All other authors report no relevant disclosures/competing interests.

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