Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Abstract

According to the 2008 World Health Organization (WHO) classification, classical Philadelphia chromosome/BCR-ABL negative chronic myeloproliferative neoplasms (MPNs) include polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF) [1]. The reported worldwide annual incidence rate of MPNs ranges from 0.44 to 5.87/10⁵, with the lowest incidence being reported in Japan and Israel [2]. These great ranges may reflect racial/geographic differences as well as differences in study design, diagnostic criteria and methods of reporting, among others. The estimated incidence rate in Europe is 0.4–2.8 × 10⁵/year for PV, 0.38–1.7 × 10⁵/year for ET and 0.1–1 × 10⁵/year for PMF [2]. There are few reliable estimates of the prevalence [2, 3]. The last is likely to be rising due to earlier diagnosis and trends towards prolonged survival [4]. The reported median age at diagnosis ranges from 65–74 years for PV, 64–73 years for ET, and 69–76 years for PMF [2].