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How common are myeloproliferative neoplasms? A systematic review and meta-analysis
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 (I(2) 97.1-99.8%) and evidence of publication bias for ET and PMF (Egger test, P = 50.007 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.
Insights
Myeloproliferative neoplasms (MPNs) are rare diseases. This review found global incidence rates for polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) but noted significant data heterogeneity.
Area of Science:
- Hematology
- Oncology
- Epidemiology
Background:
- Myeloproliferative neoplasms (MPNs) encompass a group of rare blood cancers, including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF).
- Understanding the global incidence and prevalence of MPNs is crucial for public health planning and resource allocation.
Purpose of the Study:
- To conduct a systematic review and meta-analysis of global incidence and prevalence rates for PV, ET, and PMF.
- To identify and quantify the heterogeneity and potential biases in existing MPN epidemiological data.
Main Methods:
- A comprehensive search of major electronic databases (PubMed, EMBASE, MEDLINE, Web of Science) was performed for studies reporting MPN incidence or prevalence.
- A random-effects meta-analysis was used to calculate pooled incidence rates for PV, ET, and PMF.
- Statistical assessments for heterogeneity (I² statistic) and publication bias (Egger's test) were conducted.
Main Results:
- Thirty-four studies were included, reporting a wide range of annual incidence rates for PV, ET, and PMF.
- The combined annual incidence rates per 100,000 were estimated as 0.84 for PV, 1.03 for ET, and 0.47 for PMF.
- High heterogeneity (I² > 97%) was observed across studies for all MPN types, with publication bias noted for ET and PMF.
Conclusions:
- The pooled incidence rates confirm the overall rarity of MPNs.
- Significant unexplained heterogeneity limits the ability to accurately reflect global MPN incidence and prevalence.
- Enhanced, standardized MPN registries are recommended for improved global epidemiological data collection and comparison.
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