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Comparative Value of the Novel Age-Agnostic DIPSS-R Versus the DIPSS for Prognostication in Myelofibrosis: A
Marko Lucijanić1,2,3, Davor Galušić4, Vlatka Periša5,6
1Hematology Department, University Hospital Dubrava, 10000 Zagreb, Croatia.
Background/Objectives:
The recently proposed revised Dynamic International Prognostic Scoring System (DIPSS-R) excludes age and constitutional symptoms and relies exclusively on disease-related variables. We aimed to externally validate the DIPSS-R and to characterize how it reclassifies patients relative to the DIPSS.
Methods:
We retrospectively studied 285 patients with primary or secondary myelofibrosis from seven centers. Both scores were calculable in 270 patients (148 deaths). The two systems were compared for discrimination, reclassification, calibration, and robustness across pre-specified subgroups and landmarks.
Results:
During a median follow-up of 90.3 months, median overall survival (OS) was 66.1 months (5-year OS 52.7%). The two systems showed similar discrimination (C-index 0.691 vs. 0.697; difference -0.006, 95% CI -0.046 to +0.030; p = 0.77) with no significant difference across any subgroup, fibrosis grade, driver mutation, age or sex. The DIPSS-R assigned more patients to higher-risk categories (59.3% vs. 46.3%; p < 0.001). Reclassification was discordant in 57 patients (21.1%); the DIPSS-R up-stratified 46 patients (17.0%) classified as lower-risk by the DIPSS, and these patients had significantly worse survival than concordantly lower-risk patients (5-year OS 57.7% vs. 81.7%; p < 0.001). Among transplant-eligible patients aged ≤65 years, the DIPSS-R up-stratified 26 (22.6%). The reclassification was driven by the DIPSS-R-specific variables (monocytosis, leukocytosis and thrombocytopenia). In multivariable analysis the DIPSS-R remained independently prognostic of the Charlson comorbidity index (CCI), with both retaining independent value (DIPSS-R: HR 1.96 per category, 95% CI 1.58-2.44, p < 0.001; CCI: HR 1.18 per point, 95% CI 1.06-1.32, p = 0.003). In secondary myelofibrosis the DIPSS-R discriminated comparably to the disease-specific MYSEC-PM (C-index 0.659 vs. 0.698; p = 0.44).
Conclusions:
The DIPSS-R matches the DIPSS in discrimination while identifying additional adverse-risk patients, most relevantly transplant-eligible younger patients, who would otherwise be considered lower-risk by the age-containing DIPSS; in secondary myelofibrosis it performs comparably to the disease-specific MYSEC-PM.
