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Myeloproliferative neoplasms: trends in incidence, prevalence and survival in Norway
Author(s) -
Roaldsnes Christina,
Holst René,
Frederiksen Henrik,
Ghanima Waleed
Publication year - 2017
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.12788
Subject(s) - medicine , essential thrombocythemia , incidence (geometry) , relative survival , epidemiology , polycythemia vera , myelofibrosis , cancer registry , mortality rate , demography , bone marrow , physics , sociology , optics
Background Polycythemia vera ( PV ), essential thrombocythemia ( ET ) and myelofibrosis ( MF ) are clonal disorders collectively named as myeloproliferative neoplasms ( MPN ). Published data on epidemiology of MPN after the discovery of the JAK 2 mutation and the 2008 WHO classifications are scarce. We aimed to study the incidence rates, prevalence and survival of MPN in Norway during the period 1993–2012. Method We identified 2453 persons diagnosed with MPN from the Cancer Registry of Norway between 1993 and 2012. We report age‐adjusted incidence rates, prevalence, relative survival and standardised mortality rates. Results The overall age‐adjusted yearly incidence rate of PV increased from 0.4/10⁵ to 0.7/10⁵, ET increased from 0.3/10⁵ to 1.0/10⁵ and MF from 0.2/10⁵ to 0.5/10⁵. Prevalence of PV , ET and MF was 9.2, 8.6 and 3.0 per 10⁵ inhabitants, respectively. The five‐year relative survival ( RS ) of ET and PV was slightly reduced with no improvement. The five‐year RS of MF was 58.1% (2008–2012). Standardised mortality rate ( SMR ) was 1.9 (95% CI 1.2 – 2.7). Conclusions The incidence rates of ET , PV and MF doubled and tripled during the years 2007–2012 as compared to 1995–2006. This increment in incidence rates may be related to identification of the JAK 2 mutation and the derived 2008 WHO guidelines for MPN . The RS was only slightly reduced in PV and ET , but was substantially reduced in MF .