
According to Utsav Joshi and collaborating researchers, it is unclear whether patients with primary myelofibrosis (PMF) have the increased risk for second primary malignancy (SPM) seen in other BCR-ABL-negative myeloproliferative diseases (MPD). In a study presented at the 2022 American Society of Clinical Oncology (ASCO) Annual Meeting, they reported that patients with PMF indeed have a higher risk for developing an SPM—especially leukemia and lymphoma.
Their study was based on 5,273 patients from the Surveillance, Epidemiology, and End Results (SEER) database with a diagnosis of PMF between 2009 and 2018. Any subsequent malignancy that presented after at least one year was classified as a SPM.
Among the study’s cohort, 342 patients (6.4%) developed a SPM. The authors calculated that SPM occurred at a standardized incidence ratio (SIR) of 1.97 (95% confidence interval [CI], 1.77–2.18; p <0.05) and with an absolute excess risk (AER) of 151.87 per 10,000 people. Notably, the incidences of melanoma (SIR = 1.96; 95% CI, 1.14–3.14; p <0.05), lymphoma (SIR = 3.45; 95% CI, 2.31–4.96; p <0.05), and leukemia (SIR = 26.87; 95% CI, 22.69–31.59; p <0.05) were significantly higher than other malignancies. Conversely, there were no statistically significant differences in SIR based on sex, race, marital status, follow-up duration, and receipt of chemotherapy.
Finally, SPM risk was significantly higher for patients aged ≤60 years (SIR = 2.34; 95% CI, 1.89–2.86) compared to patients aged >60 years (SIR = 1.86; 95% CI, 1.65–2.10; p = 0.01), as well as for patients with PMF diagnoses after 2009 (SIR = 2.64; 95% CI, 2.26–3.07) compared to during or before 2009 (SIR = 1.61; 95% CI, 1.40–1.85; p <0.001).
The researchers described an increased risk for SPM in patients with PMF, comparable to other MPD. In closing, the authors advanced the impact of the 2011 approval of ruxolitinib as a topic for further SPM research.