Mature survival data for 176 patients younger than 60 years with primary myelofibrosis diagnosed between 1976 and 2005: evidence for survival gains in recent years

Sergio Siragusa, Animesh Pardanani, Curtis A. Hanson, Susan M. Schwager, Kebede Hussein, Jocelin Huang, Rakhee Vaidya, Ayalew Tefferi

Risultato della ricerca: Article

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Abstract

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
Lingua originaleEnglish
pagine (da-a)1114-1119
Numero di pagine6
RivistaMayo Clinic Proceedings
Volume84
Stato di pubblicazionePublished - 2009

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Primary Myelofibrosis
Survival
Therapeutic Uses
Survival Rate
Retrospective Studies

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  • Medicine(all)

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Mature survival data for 176 patients younger than 60 years with primary myelofibrosis diagnosed between 1976 and 2005: evidence for survival gains in recent years. / Siragusa, Sergio; Pardanani, Animesh; Hanson, Curtis A.; Schwager, Susan M.; Hussein, Kebede; Huang, Jocelin; Vaidya, Rakhee; Tefferi, Ayalew.

In: Mayo Clinic Proceedings, Vol. 84, 2009, pag. 1114-1119.

Risultato della ricerca: Article

Siragusa, Sergio ; Pardanani, Animesh ; Hanson, Curtis A. ; Schwager, Susan M. ; Hussein, Kebede ; Huang, Jocelin ; Vaidya, Rakhee ; Tefferi, Ayalew. / Mature survival data for 176 patients younger than 60 years with primary myelofibrosis diagnosed between 1976 and 2005: evidence for survival gains in recent years. In: Mayo Clinic Proceedings. 2009 ; Vol. 84. pagg. 1114-1119.
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abstract = "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",
keywords = "myelofibrosis, survival",
author = "Sergio Siragusa and Animesh Pardanani and Hanson, {Curtis A.} and Schwager, {Susan M.} and Kebede Hussein and Jocelin Huang and Rakhee Vaidya and Ayalew Tefferi",
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T1 - Mature survival data for 176 patients younger than 60 years with primary myelofibrosis diagnosed between 1976 and 2005: evidence for survival gains in recent years

AU - Siragusa, Sergio

AU - Pardanani, Animesh

AU - Hanson, Curtis A.

AU - Schwager, Susan M.

AU - Hussein, Kebede

AU - Huang, Jocelin

AU - Vaidya, Rakhee

AU - Tefferi, Ayalew

PY - 2009

Y1 - 2009

N2 - 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

AB - 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

KW - myelofibrosis, survival

UR - http://hdl.handle.net/10447/55582

M3 - Article

VL - 84

SP - 1114

EP - 1119

JO - Mayo Clinic Proceedings

JF - Mayo Clinic Proceedings

SN - 0025-6196

ER -