In the past 20 years, management of primary myelofibrosis (PMF) has incorporated new treatment approaches, but survival benefits have not been confirmed in controlled studies. This retrospective study includes 176 consecutive patients younger than age 60 years in whom PMF was diagnosed during a 30-year period (1976-2005). Median age at diagnosis was 50 years (range, 18-59 years), and 98 patients (55%) were men. At the time of this report, 99 patients (56%) had died; the 77 surviving patients were followed up for a median of 8 years (range, 4-24 years). Overall median survival was 9.2 years, and 15- and 20-year survival rates were 32% and 20%, respectively. According to the Dupriez Prognostic Scoring System (PSS), median survivals were 12.7, 4.8, and 2.4 years in low- (n=117), intermediate- (n=44) and high- (n=15) risk patients (P<.001). According to the International PSS, median survivals were 13.4, 9.7, 3.3, and 2.4 years in low- (n=76), intermediate-1 (n=50), intermediate-2 (n=29), and high-risk patients (n=8; P<.001). To examine the effect of decade of diagnosis on survival, we divided study patients into 3 groups by year of diagnosis: 1976-1985 (n=36), 1986-1995 (n=45), and 1996-2005 (n=95). The corresponding median survivals were 4.8, 7.3, and "not reached" (P=.003), and the difference in survival was significant during multivariable analysis that included risk scores according to the aforementioned PSSs and age as covariates. The improvement in survival in recent years was most apparent in patients with high/intermediate-risk disease (P<.002), not in those with low-risk disease (P=.42). These observations are encouraging and suggest a salutary effect from modern therapeutic approaches in PMF.
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