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Improving survival trends in primary myelofibrosis: an international study
Francisco Cervantes1, Brigitte Dupriez, Francesco Passamonti
1Hospital Clínic, Institut d’Investigació Biomèdica August Pi i Sunyer, University of Barcelona, Spain. fcervan@clinic.ub.es
Purpose:
Despite the lack of major improvements in the treatment of primary myelofibrosis (PMF), there are recent indications that the survival of patients might have increased over the years. This study was aimed at ascertaining whether survival prolongation has actually occurred in PMF.
Patients And Methods:
A total of 802 patients diagnosed with PMF in four European countries were compared for the presentation of features and survival according to the diagnostic periods 1980 to 1995 (n = 434) and 1996 to 2007 (n = 368); relative survival was estimated for the two groups.
Results:
Patients diagnosed between 1996 and 2007 more often had constitutional symptoms (31% v 23%) but a lower incidence of marked anemia (31% v 39%), leukocytosis greater than 25 × 10(9)/L (9% v 13%), and blood blasts (27% v 33%); risk distribution was comparable between the two groups. Median survival was 4.6 years (95% CI, 4.0 to 5.1) for patients from 1980 to 1995 and 6.5 years (95% CI, 5.5 to 7.4) for patients from 1996 to 2007 (P < .001). The latter group of patients showed improved relative survival, especially for women, patients younger than age 65 years, and patients with low or intermediate-1-risk disease. Rates of PMF-attributable mortality at 5 and 10 years were significantly lower in the second period; this reduction in disease-specific mortality occurred across all patient subgroups, except in intermediate-2-risk or high-risk patients.
Conclusion:
Survival of PMF is steadily improving, except in patients in poor-risk categories. This observation must be taken into account at the time of evaluating the survival impact of newer therapies for PMF, which are currently being tested in these patient subpopulations.
Insights
Survival for primary myelofibrosis (PMF) patients has significantly improved, particularly for those diagnosed more recently. However, this survival benefit has not extended to all risk groups, highlighting areas for future therapeutic focus.
Area of Science:
- Hematology
- Oncology
- Epidemiology
Background:
- Primary myelofibrosis (PMF) is a serious myeloproliferative neoplasm.
- Treatment advancements for PMF have been limited.
- Recent trends suggest potential improvements in patient survival.
Purpose of the Study:
- To investigate whether patient survival has indeed increased over time for primary myelofibrosis.
- To compare survival rates between different diagnostic periods.
Main Methods:
- Analysis of 802 primary myelofibrosis patients across four European countries.
- Comparison of patient features and survival between two diagnostic periods: 1980-1995 (n=434) and 1996-2007 (n=368).
- Estimation of relative survival rates for both patient cohorts.
Main Results:
- Median survival increased from 4.6 years (1980-1995) to 6.5 years (1996-2007).
- Improved survival observed in the later diagnostic period, especially for women, younger patients (<65 years), and those with low/intermediate-1 risk.
- PMF-attributable mortality rates at 5 and 10 years were significantly lower in the 1996-2007 group, except for intermediate-2/high-risk patients.
Conclusions:
- Overall survival for primary myelofibrosis patients is improving.
- Survival gains are not uniform across all risk categories, with poor-risk patients not showing similar improvements.
- Future evaluations of new PMF therapies should consider these survival trends and focus on underrepresented high-risk patient groups.
