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Validation and comparison of contemporary prognostic models in primary myelofibrosis: analysis based on 334 patients
Ayalew Tefferi1, Jocelin Huang, Susan Schwager
1Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA. tefferi.ayalew@mayo.edu
Background:
Survival in primary myelofibrosis (PMF) is predicted by several prognostic scoring systems (PSSs); the most widely recognized is that of Dupriez. Two other PSSs, Cervantes and Mayo, were recently reported as being more useful in younger patients. The current study compares these 3 PSSs among all age groups.
Methods:
The Mayo Clinic PMF database was queried to identify a consecutive series of patients in whom pretreatment bone marrow and complete blood count (CBC), obtained within 6 months of diagnosis, were available for review.
Results:
Among 334 study patients (median age, 57 years), median survival was 70 months. Multivariable analysis of all 6 adverse prognostic factors utilized in the aforementioned PSSs (ie, hemoglobin <10 g/dL, leukocyte count <4 or >30 x 10(9)/L, constitutional symptoms, circulating blasts > or = 1%, platelet count <100 x 10(9)/L, absolute monocyte count > or = 1 x 10(9)/L) identified all but platelet count as being significant. The Mayo PSS, which is based on the 4 CBC-derived parameters (ie, hemoglobin, platelet, leukocyte, and monocyte counts), displayed a better hazard ratio profile compared with the other 2 PSSs in identifying long-lived patients as well as delineating intermediate-risk disease category. The latter effect was even more pronounced in patients younger than age 60 years.
Conclusions:
The Mayo PSS for survival in PMF is an objective CBC-derived prognostic model that might be superior in its performance over that of either the Dupriez and Cervantes PSSs.
Insights
The Mayo prognostic scoring system (PSS) for primary myelofibrosis (PMF) may outperform Dupriez and Cervantes PSSs. This CBC-derived model better identifies long-term survival and intermediate-risk disease across all ages.
Area of Science:
- Hematology
- Oncology
- Clinical Prognostics
Background:
- Primary myelofibrosis (PMF) survival is assessed using prognostic scoring systems (PSSs).
- The Dupriez PSS is widely recognized, while Cervantes and Mayo PSSs show utility in younger patients.
- This study compares the predictive performance of Dupriez, Cervantes, and Mayo PSSs across all age groups.
Purpose of the Study:
- To compare the effectiveness of three prognostic scoring systems (Dupriez, Cervantes, Mayo) for predicting survival in primary myelofibrosis.
- To evaluate PSS performance across different age demographics.
- To identify the most accurate PSS for stratifying PMF patient risk.
Main Methods:
- Utilized the Mayo Clinic Primary Myelofibrosis database for patient selection.
- Included consecutive patients with available pretreatment bone marrow and complete blood count (CBC) data within six months of diagnosis.
- Performed multivariable analysis of prognostic factors including hemoglobin, leukocyte count, constitutional symptoms, circulating blasts, platelet count, and absolute monocyte count.
Main Results:
- Analyzed 334 PMF patients with a median survival of 70 months.
- Multivariable analysis identified hemoglobin, leukocyte count, constitutional symptoms, and circulating blasts as significant prognostic factors, excluding platelet count.
- The Mayo PSS, based on CBC parameters (hemoglobin, leukocyte, monocyte counts), demonstrated a superior hazard ratio profile for identifying long-lived patients and intermediate-risk disease, particularly in patients under 60.
Conclusions:
- The Mayo PSS is an objective, CBC-derived prognostic model for primary myelofibrosis survival.
- The Mayo PSS may offer superior performance compared to the Dupriez and Cervantes PSSs.
- This model aids in risk stratification for PMF patients across various age groups.