Contributions
Abstract: EP1114
Type: E-Poster Presentation
Session title: Myeloproliferative neoplasms - Clinical
Background
In the recent years, prognostic models have been validated for primary myelofibrosis (PMF), initially based exclusively on clinical variables, namely the International Prognostic Scoring System-(IPSS) and the Dynamic IPSS-(DIPSS). Cytogenetics was incorporated in the most recent scores such as the DIPSS-plus. However, the applicability of PMF scoring systems in the recently recognized pre-fibrotic PMF (pre-PMF) has not been validated.
Aims
To assess the prognostic value of IPSS, DIPSS and DIPSS-plus in PMF stages: pre-PMF and overt-PMF.
Methods
Retrospective and unicentric analysis of records from patients (pts) diagnosed with PMF between January 2010 and December 2019. The diagnoses of pre-PMF and overt-PMF were reviewed according to WHO 2016 criteria. Prognostic scores were calculated considering clinical and laboratory information at diagnosis. Kaplan-Meier method were used for the analysis of progression free survival (PFS) and overall survival (OS). For multivariate analysis were included the variables with p<0.05 in the univariate analysis.
Results
We studied a total of 90 pts, with a median age of 68 years (range, 24-92 years old), 63.3% males. Forty-three (47.8%) pts were at pre-PMF stage.
The overall PFS in our cohort was 101.7 months. In addition to the variables included in the IPSS and DIPSS-plus, histological stage (HR 4.1; p=0.002), increased lactate dehydrogenase (LDH) (HR 9.7; p=0.026) and splenomegaly (HR 2.2; p=0.013) were predictors of PFS. In multivariate analysis, IPSS (HR 5.7; p<0.001), DIPSS (HR 4.5; p<0.001) and DIPSS-plus (HR 4.1; p=0.009) remained predictors of PFS. However, after sub-analysis for the histological stage, all the three scores were predictors of PFS in overt-PMF (HR 7.4, p=0.001; HR 6.8, p<0.001; HR 3.4, p=0.022, respectively) but not in pre-PMF (HR 3.1, p=0.202; HR 6.7, p=0.132; HR 2.5, p=0.237, respectively).
The overall OS was estimated in 124.6 months. Again, beyond the variables included in IPSS and DIPSS-plus, the histological stage (HR 6.2; p=0.001), elevated LDH (HR 8.8; p=0.033) and splenomegaly (HR 3.3 p=0.010) were also predictors of OS. In multivariate analysis, IPSS (HR 6.2; p<0.001), DIPSS (HR 4.5; p<0.001) and DIPSS-plus (HR 4.1; p=0.009) persisted as predictors of OS. However, upon sub-analysis for the histological stage, the aforementioned scores kept as predictors of OS in overt-PMF (HR 7.6, p=0.001; HR 6.1, p<0.001; HR 4.1, p=0.019, respectively) but not in pre-PMF (HR 3.1, p=0.200; HR 3.4, p=0.382; HR 7.9, p=0.988, respectively).
Conclusion
In our cohort, IPSS, DIPSS and DIPSS-plus were independent predictors of PFS and OS in overt-PMF, but did not in pre-PMF. These findings identify a flaw in prognostic stratification applying the available scoring systems and alarms for the need of validated scores specifically for pre-PMF pts.
Keyword(s): Myelofibrosis, Prognosis
Abstract: EP1114
Type: E-Poster Presentation
Session title: Myeloproliferative neoplasms - Clinical
Background
In the recent years, prognostic models have been validated for primary myelofibrosis (PMF), initially based exclusively on clinical variables, namely the International Prognostic Scoring System-(IPSS) and the Dynamic IPSS-(DIPSS). Cytogenetics was incorporated in the most recent scores such as the DIPSS-plus. However, the applicability of PMF scoring systems in the recently recognized pre-fibrotic PMF (pre-PMF) has not been validated.
Aims
To assess the prognostic value of IPSS, DIPSS and DIPSS-plus in PMF stages: pre-PMF and overt-PMF.
Methods
Retrospective and unicentric analysis of records from patients (pts) diagnosed with PMF between January 2010 and December 2019. The diagnoses of pre-PMF and overt-PMF were reviewed according to WHO 2016 criteria. Prognostic scores were calculated considering clinical and laboratory information at diagnosis. Kaplan-Meier method were used for the analysis of progression free survival (PFS) and overall survival (OS). For multivariate analysis were included the variables with p<0.05 in the univariate analysis.
Results
We studied a total of 90 pts, with a median age of 68 years (range, 24-92 years old), 63.3% males. Forty-three (47.8%) pts were at pre-PMF stage.
The overall PFS in our cohort was 101.7 months. In addition to the variables included in the IPSS and DIPSS-plus, histological stage (HR 4.1; p=0.002), increased lactate dehydrogenase (LDH) (HR 9.7; p=0.026) and splenomegaly (HR 2.2; p=0.013) were predictors of PFS. In multivariate analysis, IPSS (HR 5.7; p<0.001), DIPSS (HR 4.5; p<0.001) and DIPSS-plus (HR 4.1; p=0.009) remained predictors of PFS. However, after sub-analysis for the histological stage, all the three scores were predictors of PFS in overt-PMF (HR 7.4, p=0.001; HR 6.8, p<0.001; HR 3.4, p=0.022, respectively) but not in pre-PMF (HR 3.1, p=0.202; HR 6.7, p=0.132; HR 2.5, p=0.237, respectively).
The overall OS was estimated in 124.6 months. Again, beyond the variables included in IPSS and DIPSS-plus, the histological stage (HR 6.2; p=0.001), elevated LDH (HR 8.8; p=0.033) and splenomegaly (HR 3.3 p=0.010) were also predictors of OS. In multivariate analysis, IPSS (HR 6.2; p<0.001), DIPSS (HR 4.5; p<0.001) and DIPSS-plus (HR 4.1; p=0.009) persisted as predictors of OS. However, upon sub-analysis for the histological stage, the aforementioned scores kept as predictors of OS in overt-PMF (HR 7.6, p=0.001; HR 6.1, p<0.001; HR 4.1, p=0.019, respectively) but not in pre-PMF (HR 3.1, p=0.200; HR 3.4, p=0.382; HR 7.9, p=0.988, respectively).
Conclusion
In our cohort, IPSS, DIPSS and DIPSS-plus were independent predictors of PFS and OS in overt-PMF, but did not in pre-PMF. These findings identify a flaw in prognostic stratification applying the available scoring systems and alarms for the need of validated scores specifically for pre-PMF pts.
Keyword(s): Myelofibrosis, Prognosis