Risk of second primary malignancies and survival of adult patients with polycythemia vera: A United States population-based retrospective study

Abstract

Although the median survival in polycythemia vera (PV) is 14 years, mortality is higher than in an age- and sex-matched population. This study included 3941 PV patients diagnosed between 2000–2012 from Surveillance, Epidemiology and End Results (SEER) 13 registry to determine 5-year survival and the incidence of second primary malignancies (SPM). The actuarial 5 year survival in the overall cohort was 79.5%. The cumulative incidence of SPM was 13.1% at 10 years. SPMs occurred at a standardized incidence ratio (SIR) of 1.29 (95% CI = 1.16–1.43; p < 0.001) with an absolute excess risk (AER) of 42.49 per 10 000 population. A significantly higher risk was noted for acute myeloid leukemia (SIR = 12.24; 95% CI = 8.17–17.8; p-value < 0.001) and chronic myeloid leukemia (SIR = 10.66; 95% CI = 3.75–19.6; p-value < 0.001). Patients with PV are at a high risk of SPM and leukemic transformation, which may compromise long-term survival.

Keywords::

• Polycythemia vera
• second primary malignancy
• leukemic transformation
• survival

Acknowledgment

The abstract of this paper was accepted for online publication as a proceeding of 2015 American Society of Clinical Oncology annual meeting held in Chicago from May 29 to June 2, 2015.

This work was supported in part by the University of Nebraska Medical Center, College of Medicine, Physician-Scientist Training Program Grant 2015–2016.

This study utilized the SEER 13 database, available from:Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: Incidence - SEER 13 Regs Research Data, Nov 2014 Sub (1992–2012) <Katrina/Rita Population Adjustment> - Linked To County Attributes - Total U.S., 1969–2013 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2015, based on the November 2014 submission. The interpretation of this data is the sole responsibility of the authors. The authors acknowledge the efforts of Surveillance Research Program, National Cancer Institute and the SEER program tumor registries in the creation of the SEER database.

Potential conflict of interest

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Supplementary material available online

Supplementary Table I showing further data.