Purpose: Recent diagnostic and cancer reporting changes influencing myeloproliferative neoplasms (MPNs) encourage the assessment of trends and examination of the recently identified MPN subtypes: polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), across the age continuum by race and ethnicity.
Methods: Surveillance, Epidemiology, and End Results data provided MPN incidence data since 1973 and MPN subtype data since 2001. Joinpoint regression estimated annual percent changes. Poisson regression estimated risk ratios.
Results: The 2005 JAK2 V617F discovery and the 2008 WHO diagnostic guideline for the JAK2 V617F mutation coincide with a 31 % increase in ET and a 21 % decrease in PV incidence rates. We found that younger women had a 13-33 % higher ET risk and that women under the age of 34 had a 58 % higher PMF risk, relative to men. Blacks, aged 35-49 with a higher ET risk, also had a 69 % higher PMF risk relative to whites.
Conclusion: Demographic characteristic of ET and PMF patients may be useful for improving risk prediction and informing clinical screening and treatment strategies. Changing guidelines, new discoveries, and in-depth analysis of a large population-based study have implications for accurately identifying incident cases of MPNs, MPN subgroups, and health resource planning.
Keywords: Essential thrombocythemia; Incidence; Myeloproliferative neoplasm; Polycythemia vera; Primary myelofibrosis; Trends.