DIPSS Prognosis in Myelofibrosis
Estimate prognosis in myelofibrosis.
This prognostic scoring system for primary myelofibrosis resulted from data from 1054 consecutively diagnosed patients with PMF from 1980 to 2007. Patients were identified at 7 American and European institutions. Overall median survival was 5.7 years and only 5 patients in the cohort underwent allogeneic stem cell transplantation. Based on the presence of the 5 identified prognostic parameters, 4 risk groups were identified with median survivals ranging from 27 months (high-risk) to 135 months (low-risk). Cytogenetic abnormalities were also associated with shorter survival but did not appear to contribute to the overall model, potentially due to the limits in sampling cytogenetics in all individuals studied.
Recently, a dynamic prognostic model was established to determine whether the acquisition of new risk factors modified the outcome. The same 5 variables established above had significant impact on survival when analyzed as time-dependent covariates in 525 primary MF patients. The acquisition of anemia appeared to affect survival to a greater extent than other factors. The Dynamic International Prognostic Scoring System (DIPSS) was established to enable ongoing prognostic assessment of PMF patients at any point during their clinical follow-up.
The MF scoring system was proposed and validated by Cervantes et al.
The DIPSS was proposed and validated by Passamonti et al.
Prognosis depends on sum of 5 factors:
- If score is 0: Patient is considered "low risk" according to the scoring system. Median survival was not reached (median follow-up 3.3 years). If a patient changes risk category to intermediate-1, the hazard ratio for increased mortality is HR=4.13.
- If score is 1-2: Patient is considered "intermediate-1 risk" according to the scoring system. Median survival was 14.1 years (from the time of diagnosis). If a patient changes risk category to intermediate-2, the hazard ratio for increased mortality is HR=4.61.
- If score is 3-4: Patient is considered "intermediate-2 risk" according to the scoring system. Median survival was 4 years (from the time of diagnosis). If a patient changes risk category to high-risk, the hazard ratio for increased mortality is HR=2.54.
- If score is 5 or more: Patient is considered "high risk" according to the scoring system. Median survival was 1.5 years (from the time of diagnosis)
Cervantes et al.
Passamonti et al.