title: 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.
authors.affiliation: a Department of Internal Medicine , Creighton University Medical Center , Omaha , NE , USA.
authors.forename: Nabin
authors.initials: N
authors.lastname: Khanal
authors.affiliation: b Department of Medicine , University at Tennessee , TN , USA.
authors.forename: Smith
authors.initials: S
authors.lastname: Giri
authors.affiliation: a Department of Internal Medicine , Creighton University Medical Center , Omaha , NE , USA.
authors.forename: Smrity
authors.initials: S
authors.lastname: Upadhyay
authors.affiliation: c College of Public Health, Department of Biostatistics , University of Nebraska Medical Center , NE , USA.
authors.forename: Valerie K
authors.initials: VK
authors.lastname: Shostrom
authors.affiliation: d Department of Medicine , Reading Health System, Reading , MA , USA.
authors.forename: Ranjan
authors.initials: R
authors.lastname: Pathak
authors.affiliation: e Division of Hematology and Oncology, Department of Internal Medicine , University of Nebraska Medical Center , NE , USA.
authors.forename: Vijaya Raj
authors.initials: VR
authors.lastname: Bhatt
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality*; D011087:Polycythemia Vera / Q000453:epidemiology / Q000401:mortality*; D011159:Population Surveillance; D012189:Retrospective Studies; D012306:Risk; D018426:SEER Program; D014481:United States / Q000453:epidemiology
keywords: Polycythemia vera; leukemic transformation; second primary malignancy; survival