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Patterns of survival among patients with myeloproliferative neoplasms diagnosed in Sweden from 1973 to 2008: a
Malin Hultcrantz1, Sigurdur Yngvi Kristinsson, Therese M-L Andersson
1Karolinska University Hospital, Stockholm, Sweden. malin.hultcrantz@karolinska.se
Purpose:
Reported survival in patients with myeloproliferative neoplasms (MPNs) shows great variation. Patients with primary myelofibrosis (PMF) have substantially reduced life expectancy, whereas patients with polycythemia vera (PV) and essential thrombocythemia (ET) have moderately reduced survival in most, but not all, studies. We conducted a large population-based study to establish patterns of survival in more than 9,000 patients with MPNs.
Patients And Methods:
We identified 9,384 patients with MPNs (from the Swedish Cancer Register) diagnosed from 1973 to 2008 (divided into four calendar periods) with follow-up to 2009. Relative survival ratios (RSRs) and excess mortality rate ratios were computed as measures of survival.
Results:
Patient survival was considerably lower in all MPN subtypes compared with expected survival in the general population, reflected in 10-year RSRs of 0.64 (95% CI, 0.62 to 0.67) in patients with PV, 0.68 (95% CI, 0.64 to 0.71) in those with ET, and 0.21 (95% CI, 0.18 to 0.25) in those with PMF. Excess mortality was observed in patients with any MPN subtype during all four calendar periods (P < .001). Survival improved significantly over time (P < .001); however, the improvement was less pronounced after the year 2000 and was confined to patients with PV and ET.
Conclusion:
We found patients with any MPN subtype to have significantly reduced life expectancy compared with the general population. The improvement over time is most likely explained by better overall clinical management of patients with MPN. The decreased life expectancy even in the most recent calendar period emphasizes the need for new treatment options for these patients.
Insights
Patients with myeloproliferative neoplasms (MPNs) have reduced life expectancy. Survival has improved over time, particularly for polycythemia vera (PV) and essential thrombocythemia (ET), but new treatments are still needed.
Area of Science:
- Hematology
- Oncology
- Epidemiology
Background:
- Myeloproliferative neoplasms (MPNs) encompass a group of blood cancers with variable survival rates.
- Primary myelofibrosis (PMF) is associated with significantly reduced life expectancy, while polycythemia vera (PV) and essential thrombocythemia (ET) show moderately reduced survival.
Purpose of the Study:
- To conduct a large population-based study to determine survival patterns in over 9,000 patients diagnosed with MPNs.
- To analyze survival variations across different MPN subtypes and diagnostic periods.
Main Methods:
- Utilized data from the Swedish Cancer Register for 9,384 MPN patients diagnosed between 1973 and 2008.
- Calculated relative survival ratios (RSRs) and excess mortality rate ratios for survival analysis, with follow-up until 2009.
Main Results:
- All MPN subtypes exhibited considerably lower survival than the general population, with 10-year RSRs of 0.64 for PV, 0.68 for ET, and 0.21 for PMF.
- Excess mortality was present across all MPN subtypes and diagnostic periods.
- Survival significantly improved over time, though this trend slowed after 2000 and was primarily observed in PV and ET patients.
Conclusions:
- Patients with any MPN subtype face significantly reduced life expectancy compared to the general population.
- Improvements in survival are likely due to enhanced clinical management.
- The persistent decrease in life expectancy, even in recent periods, highlights the urgent need for novel therapeutic strategies for MPN patients.
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