2015 ESMO指南:费城染色体阴性慢性骨髓增生性肿瘤的诊断、治疗和随访
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). 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. 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. There are few reliable estimates of the prevalence. The last is likely to be rising due to earlier diagnosis and trends towards prolonged survival. The reported median age at diagnosis ranges from 65–74 years for PV, 64–73 years for ET, and 69–76 years for PMF.
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