Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union

Odile Moulard, Jyotsna Mehta, Jon Fryzek, Robert Olivares, Usman Iqbal, Ruben A. Mesa

Research output: Contribution to journalArticle

67 Citations (Scopus)

Abstract

Background: Primary myelofibrosis (PMF), essential thrombocythemia (ET), and polycythemia vera (PV) are BCR ABL-negative myeloproliferative neoplasms (MPN). Published epidemiology data are scarce, and multiple sources are needed to assess the disease burden. Methods: We assembled the most recent information available on the incidence and prevalence of myelofibrosis (MF), ET, and PV by conducting a structured and exhaustive literature review of the published peer-reviewed literature in EMBASE 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. Results: Eleven articles identified from EMBASE, three online hematology or oncology registries, and two Web-based databases or reports were used to summarize epidemiological estimates for MF, PV, and ET. The incidence rate of MF ranged from 0.1 per 100 000 per year to 1 per 100 000 per year. Among the various registries, the incidence of PV ranged from 0.4 per 100 000 per year to 2.8 per 100 000 per year, while the literature estimated the range of PV incidence to be 0.68 per 100 000 to 2.6 per 100 000 per year. The estimated incidence of ET was between 0.38 per 100 000 per year and 1.7 per 100 000 per year. While a few studies reported on the MPNs' 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). Conclusion: There is a wide variation in both prevalence and incidence estimates observed across European data sources. Carefully designed studies, with standardized definitions of MPNs and complete ascertainment of patients including both primary and secondary MFs, 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.

Original languageEnglish (US)
Pages (from-to)289-297
Number of pages9
JournalEuropean Journal of Haematology
Volume92
Issue number4
DOIs
StatePublished - 2014

Fingerprint

Essential Thrombocythemia
Polycythemia Vera
Primary Myelofibrosis
European Union
Epidemiology
Registries
Incidence
Population
Insurance Carriers
Health Planning
Cost of Illness
Peer Review
Information Storage and Retrieval
Hematology
Administrative Personnel
Documentation
Neoplasms
Public Health
Databases
Delivery of Health Care

Keywords

  • Epidemiology
  • Essential thrombocythemia
  • Europe
  • Myelofibrosis
  • Polycythemia vera

ASJC Scopus subject areas

  • Hematology
  • Medicine(all)

Cite this

Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union. / Moulard, Odile; Mehta, Jyotsna; Fryzek, Jon; Olivares, Robert; Iqbal, Usman; Mesa, Ruben A.

In: European Journal of Haematology, Vol. 92, No. 4, 2014, p. 289-297.

Research output: Contribution to journalArticle

Moulard, Odile ; Mehta, Jyotsna ; Fryzek, Jon ; Olivares, Robert ; Iqbal, Usman ; Mesa, Ruben A. / Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union. In: European Journal of Haematology. 2014 ; Vol. 92, No. 4. pp. 289-297.
@article{05d2d6e8472c43729c91c5fd9a7c50fa,
title = "Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union",
abstract = "Background: Primary myelofibrosis (PMF), essential thrombocythemia (ET), and polycythemia vera (PV) are BCR ABL-negative myeloproliferative neoplasms (MPN). Published epidemiology data are scarce, and multiple sources are needed to assess the disease burden. Methods: We assembled the most recent information available on the incidence and prevalence of myelofibrosis (MF), ET, and PV by conducting a structured and exhaustive literature review of the published peer-reviewed literature in EMBASE 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. Results: Eleven articles identified from EMBASE, three online hematology or oncology registries, and two Web-based databases or reports were used to summarize epidemiological estimates for MF, PV, and ET. The incidence rate of MF ranged from 0.1 per 100 000 per year to 1 per 100 000 per year. Among the various registries, the incidence of PV ranged from 0.4 per 100 000 per year to 2.8 per 100 000 per year, while the literature estimated the range of PV incidence to be 0.68 per 100 000 to 2.6 per 100 000 per year. The estimated incidence of ET was between 0.38 per 100 000 per year and 1.7 per 100 000 per year. While a few studies reported on the MPNs' 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). Conclusion: There is a wide variation in both prevalence and incidence estimates observed across European data sources. Carefully designed studies, with standardized definitions of MPNs and complete ascertainment of patients including both primary and secondary MFs, 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.",
keywords = "Epidemiology, Essential thrombocythemia, Europe, Myelofibrosis, Polycythemia vera",
author = "Odile Moulard and Jyotsna Mehta and Jon Fryzek and Robert Olivares and Usman Iqbal and Mesa, {Ruben A.}",
year = "2014",
doi = "10.1111/ejh.12256",
language = "English (US)",
volume = "92",
pages = "289--297",
journal = "European Journal of Haematology",
issn = "0902-4441",
publisher = "Wiley-Blackwell",
number = "4",

}

TY - JOUR

T1 - Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union

AU - Moulard, Odile

AU - Mehta, Jyotsna

AU - Fryzek, Jon

AU - Olivares, Robert

AU - Iqbal, Usman

AU - Mesa, Ruben A.

PY - 2014

Y1 - 2014

N2 - Background: Primary myelofibrosis (PMF), essential thrombocythemia (ET), and polycythemia vera (PV) are BCR ABL-negative myeloproliferative neoplasms (MPN). Published epidemiology data are scarce, and multiple sources are needed to assess the disease burden. Methods: We assembled the most recent information available on the incidence and prevalence of myelofibrosis (MF), ET, and PV by conducting a structured and exhaustive literature review of the published peer-reviewed literature in EMBASE 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. Results: Eleven articles identified from EMBASE, three online hematology or oncology registries, and two Web-based databases or reports were used to summarize epidemiological estimates for MF, PV, and ET. The incidence rate of MF ranged from 0.1 per 100 000 per year to 1 per 100 000 per year. Among the various registries, the incidence of PV ranged from 0.4 per 100 000 per year to 2.8 per 100 000 per year, while the literature estimated the range of PV incidence to be 0.68 per 100 000 to 2.6 per 100 000 per year. The estimated incidence of ET was between 0.38 per 100 000 per year and 1.7 per 100 000 per year. While a few studies reported on the MPNs' 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). Conclusion: There is a wide variation in both prevalence and incidence estimates observed across European data sources. Carefully designed studies, with standardized definitions of MPNs and complete ascertainment of patients including both primary and secondary MFs, 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.

AB - Background: Primary myelofibrosis (PMF), essential thrombocythemia (ET), and polycythemia vera (PV) are BCR ABL-negative myeloproliferative neoplasms (MPN). Published epidemiology data are scarce, and multiple sources are needed to assess the disease burden. Methods: We assembled the most recent information available on the incidence and prevalence of myelofibrosis (MF), ET, and PV by conducting a structured and exhaustive literature review of the published peer-reviewed literature in EMBASE 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. Results: Eleven articles identified from EMBASE, three online hematology or oncology registries, and two Web-based databases or reports were used to summarize epidemiological estimates for MF, PV, and ET. The incidence rate of MF ranged from 0.1 per 100 000 per year to 1 per 100 000 per year. Among the various registries, the incidence of PV ranged from 0.4 per 100 000 per year to 2.8 per 100 000 per year, while the literature estimated the range of PV incidence to be 0.68 per 100 000 to 2.6 per 100 000 per year. The estimated incidence of ET was between 0.38 per 100 000 per year and 1.7 per 100 000 per year. While a few studies reported on the MPNs' 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). Conclusion: There is a wide variation in both prevalence and incidence estimates observed across European data sources. Carefully designed studies, with standardized definitions of MPNs and complete ascertainment of patients including both primary and secondary MFs, 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.

KW - Epidemiology

KW - Essential thrombocythemia

KW - Europe

KW - Myelofibrosis

KW - Polycythemia vera

UR - http://www.scopus.com/inward/record.url?scp=84896548818&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84896548818&partnerID=8YFLogxK

U2 - 10.1111/ejh.12256

DO - 10.1111/ejh.12256

M3 - Article

VL - 92

SP - 289

EP - 297

JO - European Journal of Haematology

JF - European Journal of Haematology

SN - 0902-4441

IS - 4

ER -