Abstract Background: Classic MPNs include Essential Thrombocythemia (ET), Polycythemia Vera (PV), Primary Myelofibrosis (PMF), and Chronic Myeloid Leukemia (CML). Epidemiology and overall outcomes in light of recent advances in JAK inhibitors and TKIs is not well characterized. Herein, we present incidence and survival outcomes trends over the past decades using SEER registry data. Methods: We retrospectively interrogated NCI's Surveillance, Epidemiology and End Result (SEER)-17 registries to analyze adult patients diagnosed with MPN ICD-O-3 codes: PV 9950/3, ET 9962/3, PMF 9961/3, CML 9875/3 during the years 2000 to 2021. Demographic data were extracted using SEER-Stat v8.4.3 software's MP-SIR session. Descriptive statistics and Kaplan-Meier survival analysis were performed using R (v4.2.2)-software. Results: A total of 63,242 (ET, n=24,172; PV, n= 23,456; PMF, n=6,131; CML, n=9,483) patients were evaluated. The overall Incidence Rate (IR) for ET, PV, PMF, and CML was 1.8, 1.7, 0.5, and 0.7 per 100,000 person-years, respectively. The incidence of all Philadelphia-negative MPNs increased with age in this population, and most cases were seen after the age of 50. The IR for ET and PV was highest in the population over age 85 (7.9 and 6.1 per 100,000 person-years, respectively). The incidence rates of PMF were highest in ages 80-85 years (2.4 per 100,000 person-years) and for CML at 75-85 years (1.5 per 100,000 person-years). Five- year Relative Survival (RS) was noted to be 93.30%, 91.60%, 86.20% and 52.40% for ET, PV, CML and PMF, respectively. Ten-year RS for ET, PV, CML, and PMF was 83.2%, 79.1%, 77.6%, and 26.50%, respectively. Five-year RS for patients living with PMF improved over the recent years, and was noted to be 50.9% in 2009, 57.4% in 2013, and 60.6% in 2016. The median overall survival was 152 months(m) (95% CI, 149-155m) for ET, 150 m (95% CI, 147-153m) for PV, 48 m (95% CI, 46-50 m) for PMF, and 202 m(95% CI 187-216 m) for CML. Overall survival was longer in women versus men in ET (median OS 168 months, 95% CI 163-172 months, versus median OS 131 months,95% CI 126-135 months, p 0.001), PMF (median OS 63 months, 95% CI 59-68 months versus 42 months, 95% CI 40-44 months, p 0.001) and CML (median OS 212 months, 95% CI 192-242 months, versus median OS 197 months, 95% CI 175-214 months, p =0.0073). In contrast, a significantly longer OS was noted in men with PV (median OS 162 months, 95% CI 157-167 months versus 136 months, 95% CI 132-140 months in women, p 0.001) Conclusions: While limited by inherent biases of a large registry data study, our study demonstrates that reported rate of MPNs in the SSER registry has increased in the recent years, likely due to improved access and wider applicability of diagnostic technology especially sequencing of driver mutations. The sex-related differences in outcomes, previously reported in smaller studies due to differences in the genetic signatures, are confirmed in this large registry study.
Arora et al. (Mon,) studied this question.