How common are the Myeloproliferative
Neoplasms: A Systematic review and Meta-Analysis.
Glen J Titmarsh1, Andrew Duncombe2, Mary Frances McMullin3, Michael O’Rorke1, Ruben Mesa4, Frank De Vocht5, Sarah Horan6, Lin Fritschi7, Mike Clarke1, Lesley A Anderson1.
1Centre
for Public Health, Queen’s University Belfast, Northern Ireland.
2Department of Haematology, University of Southampton, UK.
3Centre for Cancer Research and Cell Biology, Queen’s University Belfast, Northern Ireland.
4Mayo Clinic Cancer Centre. Arizona. USA.
5School of Social and Community Medicine. University of Bristol, UK
6School of Health Sciences. City University London, UK.
7School of Public Health, Epidemiology and Biostatistics. Curtin University. Australia
Results
Background
•
The myeloproliferative neoplasms (MPNs) are a heterogeneous
group of diseases characterised by overproduction of one or more
myeloid cell types via bone marrow stimulation.
•
Entities include;
•
Polycythaemia vera, (PV)
•
Essential thrombocythaemia (ET)
•
Primary (idiopathic) myelofibrosis (PMF)
•
The JAK2V617F mutation is central to all three disease entities.
•
These diseases possess specific genetic lesions and clinical
characteristics.
•
There are currently no definitive incidence rates available.
Pooled Crude Rate
Per 100,000 Per
Year (95% CI)
Incidence Range
per 100,000
I2
Begg’s Test
p-value
Egger’s Test
p-value
Methods
•
Embase, Medline, PubMed and Web of Science databases were
No language or other restrictions were imposed.
•
A random effects meta-analysis was undertaken to produce combined
Pooled Crude
Male Rate
Per 100,000 Per
Year (95% CI)
Pooled Crude
Female Rate
Per 100,000 Per
Year (95% CI)
incidence rates for PV, ET and PMF.
•
•
Primary
Myelofibrosis
(PMF)
0.84
1.03
0.47
2.58
(0.70 – 1.01)
(0.58 – 1.80)
(0.34 – 0.65)
(1.90 – 3.50)
0.01 to 2.61
0.21 to 2.27
0.22 to 0.99
1.15 to 4.99
99.1%
99.6%
Publication bias
97.1%
99.6%
0.674
0.210
1.000
1.000
0.237
0.007
<0.001
0.039
Gender
searched for articles reporting incidence rates for MPNs.
•
Polycythaemia
Vera (PV)
Essential
Thrombocythaemia
(ET)
Classic
Myeloproliferative
Neoplasms (MPNs)
Combined
Heterogeneity was assessed using the I2 statistic.
0.87
1.44
0.59
2.56
(0.58 – 1.30)
(0.81 – 2.53)
(0.39 – 0.90)
(1.12 – 5.81)
0.73
2.12
0.30
2.27
(0.46 – 1.15)
(1.27 – 3.54)
(0.22 – 0.42)
(0.95 – 5.42)
Time Period
Publication bias assessed using Begg-Mazumdar and Egger tests.
Pooled Crude Rate
Per 100,000 Per
Year
Pre 2005 (95% CI)
0.84
1.01
0.49
2.52
(0.70 – 1.01)
(0.58 – 1.75)
(0.34 – 0.70)
(1.91 – 3.33)
Conclusion

The included studies showed high heterogeneity, demonstrating that the reported
incidence rates are unlikely to be measuring a single, true underlying value of incidence
rates.

The pooled incidence rates reflect the rarity of MPNs in the general population.

Improved, widespread registration of MPNs would provide better information for global
comparison of the incidence of these diseases which will be relevant to clinician’s and
guide further service development and scientific research.