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Myeloproliferative neoplasms: trends in incidence, prevalence and survival in Norway
Christina Roaldsnes1, René Holst1,2, Henrik Frederiksen3
1Dept of Medicine, Ostfold Hospital, Norway.
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 JAK2 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 JAK2 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.
Insights
The incidence of myeloproliferative neoplasms (MPN), including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF), has significantly increased in Norway. Survival rates for PV and ET remain stable, while myelofibrosis shows a substantial survival reduction.
Area of Science:
- Hematology
- Oncology
- Epidemiology
Background:
- Myeloproliferative neoplasms (MPN), encompassing polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF), are clonal hematopoietic stem cell disorders.
- Limited epidemiological data exists for MPN following the discovery of the JAK2 mutation and the 2008 WHO classification updates.
- Understanding current MPN epidemiology is crucial for patient care and resource allocation.
Purpose of the Study:
- To investigate the incidence, prevalence, and survival rates of MPN in Norway.
- To analyze trends in MPN epidemiology between 1993 and 2012.
- To assess the impact of diagnostic advancements on MPN epidemiology.
Main Methods:
- Utilized data from the Cancer Registry of Norway for 2453 individuals diagnosed with MPN from 1993 to 2012.
- Calculated age-adjusted incidence rates, prevalence, relative survival (RS), and standardized mortality rates (SMR).
- Analyzed epidemiological trends over a 20-year period.
Main Results:
- Overall age-adjusted incidence rates for PV, ET, and MF doubled to tripled between 1995-2006 and 2007-2012.
- Prevalence per 10⁵ inhabitants was 9.2 for PV, 8.6 for ET, and 3.0 for MF.
- Five-year RS for ET and PV showed slight reductions with no improvement, while MF had a five-year RS of 58.1% (2008-2012).
Conclusions:
- MPN incidence rates have significantly increased, potentially linked to JAK2 mutation discovery and 2008 WHO guidelines.
- Survival for PV and ET shows minimal change, but survival for MF is substantially reduced.
- These findings highlight evolving MPN epidemiology and differential survival outcomes.
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