Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the <scp>E</scp>uropean <scp>U</scp>nion

<jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Primary myelofibrosis (<jats:styled-content style="fixed-case">PMF</jats:styled-content>), essential thrombocythemia (<jats:styled-content style="fixed-case">ET</jats:styled-content>), and polycythemia vera (<jats:styled-content style="fixed-case">PV</jats:styled-content>) are <jats:styled-content style="fixed-case">BCR ABL</jats:styled-content>‐negative myeloproliferative neoplasms (<jats:styled-content style="fixed-case">MPN</jats:styled-content>). Published epidemiology data are scarce, and multiple sources are needed to assess the disease burden.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We assembled the most recent information available on the incidence and prevalence of myelofibrosis (<jats:styled-content style="fixed-case">MF</jats:styled-content>), <jats:styled-content style="fixed-case">ET</jats:styled-content>, and <jats:styled-content style="fixed-case">PV</jats:styled-content> by conducting a structured and exhaustive literature review of the published peer‐reviewed literature in <jats:styled-content style="fixed-case">EMBASE</jats:styled-content> and by reviewing online documentation from disease registries and relevant health registries in European countries. The search was restricted to human studies written in English or French and published between January 1, 2000, and December 6, 2012.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Eleven articles identified from <jats:styled-content style="fixed-case">EMBASE</jats:styled-content>, three online hematology or oncology registries, and two Web‐based databases or reports were used to summarize epidemiological estimates for <jats:styled-content style="fixed-case">MF</jats:styled-content>,<jats:styled-content style="fixed-case"> PV</jats:styled-content>, and <jats:styled-content style="fixed-case">ET</jats:styled-content>. The incidence rate of <jats:styled-content style="fixed-case">MF</jats:styled-content> ranged from 0.1 per 100 000 per year to 1 per 100 000 per year. Among the various registries, the incidence of <jats:styled-content style="fixed-case">PV</jats:styled-content> ranged from 0.4 per 100 000 per year to 2.8 per 100 000 per year, while the literature estimated the range of <jats:styled-content style="fixed-case">PV</jats:styled-content> incidence to be 0.68 per 100 000 to 2.6 per 100 000 per year. The estimated incidence of <jats:styled-content style="fixed-case">ET</jats:styled-content> was between 0.38 per 100 000 per year and 1.7 per 100 000 per year. While a few studies reported on the <jats:styled-content style="fixed-case">MPN</jats:styled-content>s' prevalences, it is difficult to compare them as various types of prevalence were calculated (point prevalence vs. period prevalence) and standardization was made according to different populations (e.g., the world population and the European population).</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>There is a wide variation in both prevalence and incidence estimates observed across European data sources. Carefully designed studies, with standardized definitions of <jats:styled-content style="fixed-case">MPN</jats:styled-content>s and complete ascertainment of patients including both primary and secondary <jats:styled-content style="fixed-case">MF</jats:styled-content>s, should be conducted so that estimates of the population aimed to receive novel treatments for these neoplasms are better understood assist public health planning and provide valuable information about the burden of illness to policy makers, funding agencies, resource planners, healthcare insurers, and pharmaceutical manufacturers.</jats:p></jats:sec>