Trends and disparities in polycythemia vera–related mortality in the United States from 1999 to 2020.

Abstract

e22557 Background: Polycythemia Vera (PV) is a rare JAK2-mutated myeloproliferative neoplasm with a 5-year survival rate of approximately 79.5%. Patients are at an increased risk of secondary malignancies, leukemic transformation, and fatal thromboembolic events, all of which can significantly impact long-term survival. Analyzing trends in PV-related deaths is crucial for understanding these risks and improving patient outcomes. Methods: Crude mortality rates (CMRs) and age-adjusted mortality rates (AAMRs) per 100,000 population from 1999 to 2020 were extracted from the CDC Wide-Ranging Online Data for Epidemiological Research (WONDER) database using ICD-10 code D45. AAMRs were stratified by year, gender, race, age groups, place of death, and region. Annual percentage change (APC) in AAMR was calculated using Joinpoint regression (V 5.2.0, National Cancer Institute). Results: From 1999 to 2020, 21,270 deaths related to PV were reported. The AAMR declined from 0.4 in 1999 to 0.22 in 2015 (APC = -3.75%, 95% CI: -4.07% to -3.42%, p < 0.001). However, after 2015, the trend reversed, with the AAMR rising to 0.28 in 2020 (APC = 3.95%, 95% CI: 1.55% to 6.40%, p = 0.028). Throughout the study period, men had higher AAMR values (overall AAMR = 0.34) than women (overall AAMR = 0.25). Non-Hispanic White individuals had the highest overall AAMR (0.31), with a decline from 1999 to 2015 (APC = -3.74%, p < 0.001) and an increase from 2015 to 2020 (APC = 3.94%, p = 0.012). Age-related increases in mortality were evident, with the highest AAMR seen in individuals over 85 years (AAMR = 5.6). Regionally, the Midwest had the highest AAMR (0.34), followed by the West (0.32), Northeast (0.29), and South (0.23). In rural areas, the overall AAMR was higher (0.32) compared to urban areas (0.29). Urban areas saw a decline in AAMR from 1999 to 2015 (APC = -3.96%, p < 0.001), followed by an increase from 2015 to 2020 (APC = 3.86%, p = 0.017). Rural areas experienced a steady decline from 1999 to 2017 (APC = -2.82%, p < 0.001), followed by a sharp increase from 2017 to 2020 (APC = 11.38%, p = 0.003).Regarding the location of death, 41% occurred in medical facilities, 32.7% at home, 3.2% in hospices, 18.9% in nursing homes, and 4.1% in other or unknown locations. Conclusions: From 1999 to 2015, PV-related mortality declined but began to rise after 2015, continuing through 2020. Disparities in mortality were observed across gender, age, race, region, and urbanization. These findings emphasize the need for targeted interventions, tailored treatments, and further research to address the increased mortality risk associated with PV.