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.


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