Epidemiology of the Philadelphia Chromosome-Negative Classical Myeloproliferative Neoplasms

Rory M Shallis; Amer M Zeidan; Rong Wang; Nikolai A Podoltsev

doi:10.1016/j.hoc.2020.11.005

Epidemiology of the Philadelphia Chromosome-Negative Classical Myeloproliferative Neoplasms

Hematol Oncol Clin North Am. 2021 Apr;35(2):177-189. doi: 10.1016/j.hoc.2020.11.005. Epub 2021 Jan 4.

Authors

Rory M Shallis¹, Amer M Zeidan¹, Rong Wang², Nikolai A Podoltsev³

Affiliations

¹ Section of Hematology, Department of Internal Medicine, Yale University School of Medicine and Yale Cancer Center, 333 Cedar Street, P.O. Box 208028, New Haven, CT 06520-8028, USA.
² Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, 333 Cedar Street, P.O. Box 208028, New Haven, CT 06520-8028, USA.
³ Section of Hematology, Department of Internal Medicine, Yale University School of Medicine and Yale Cancer Center, 333 Cedar Street, P.O. Box 208028, New Haven, CT 06520-8028, USA. Electronic address: nikolai.podoltsev@yale.edu.

PMID: 33641862
DOI: 10.1016/j.hoc.2020.11.005

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.

Publication types

Review

MeSH terms

Humans
Male
Myeloproliferative Disorders* / epidemiology
Myeloproliferative Disorders* / genetics
Philadelphia Chromosome
Polycythemia Vera* / epidemiology
Polycythemia Vera* / genetics
Primary Myelofibrosis* / epidemiology
Primary Myelofibrosis* / genetics
Thrombocythemia, Essential* / epidemiology
Thrombocythemia, Essential* / genetics