How common are myeloproliferative neoplasms? A systematic review and meta-analysis

Glen J. Titmarsh, Andrew S. Duncombe, Mary Frances Mcmullin, Michael O'Rorke, Ruben Mesa, Frank De Vocht, Sarah Horan, Lin Fritschi, Mike Clarke, Lesley A. Anderson

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Abstract

Myeloproliferative neoplasms (MPNs) are a heterogeneous group of diseases including polycythemia vera (PV), essential thrombocythemia (ET), and primary(idiopathic) myelofibrosis (PMF). In this systematic review, we provide a comprehensive report on the incidence and prevalence of MPNs across the globe. Electronic databases (PubMed, EMBASE, MEDLINE, and Web of Science) were searched from their inception to August 2012 for articles reporting MPN incidence or prevalence rates. A random effects meta-analysis was undertaken to produce combined incidence rates for PV, ET, and PMF. Both heterogeneity and small study bias were assessed. Thirty-four studies were included. Reported annual incidence rates ranged from 0.01 to 2.61, 0.21 to 2.27, and 0.22 to 0.99 per 100,000 for PV, ET, and PMF, respectively. The combined annual incidence rates for PV, ET, and PMF were 0.84, 1.03, and 0.47 per 100,000. There was high heterogeneity across disease entities (I2 97.1-99.8%) and evidence of publication bias for ET and PMF (Egger test, P=0.007 and P≤0.001, respectively).The pooled incidence reflects the rarity of MPNs. The calculated pooled incidence rates do not reflect MPN incidence across the globe due to the high unexplained heterogeneity. Improved, widespread registration of MPNs would provide better information for global comparison of the incidence and prevalence of MPNs.

Original languageEnglish (US)
Pages (from-to)581-587
Number of pages7
JournalAmerican Journal of Hematology
Volume89
Issue number6
DOIs
StatePublished - May 2014

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ASJC Scopus subject areas

  • Hematology

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Titmarsh, G. J., Duncombe, A. S., Mcmullin, M. F., O'Rorke, M., Mesa, R., De Vocht, F., Horan, S., Fritschi, L., Clarke, M., & Anderson, L. A. (2014). How common are myeloproliferative neoplasms? A systematic review and meta-analysis. American Journal of Hematology, 89(6), 581-587. https://doi.org/10.1002/ajh.23690