Revised International Prognostic Scoring System (IPSS-R) for Myelodysplastic Syndrome (MDS)
Why Use
Can help determine whether to treat or observe, and what type of treatment, based on risk score.
When to Use
Patients newly-diagnosed with myelodysplastic syndrome.
Formula
Pearls / Pitfalls
The IPSS-R categorizes patients into 1 of 5 groups, from very low risk to very high risk, based on risk of mortality and transformation to acute myeloid leukemia (AML). This score is not dynamic and is meant to be used at the time of diagnosis only. That being said, the score does get higher with disease progression. The IPSS-R has better discrimination and is more widely used over the original IPSS (see Evidence). Data are based on untreated patients, represent statistical probabilities, and are not absolute.
Management
Very low or low risk patients can be observed and given supportive care only with monitoring of blood counts, unless they have another indication for treatment, such as symptomatic cytopenias. If these patients require treatment, they can usually be managed with low-intensity regimens such as growth factors, hypomethylating agents or lenalidomide. High or very high risk patients will usually require treatment with high-intensity therapies such as chemotherapy and allogeneic transplant. Intermediate patients can be treated with either low- or high-intensity treatments based on age, performance status and patient preference. Patients with deletion 5q and up to one other cytogenetic abnormality (as long as it is not del7q) are special cases that are very low risk and respond to lenalidomide. Treatment-related MDS is also a special category that is very high risk.
Critical Actions
Treatment decision should be individualized, and since most patients with MDS are older, comorbid conditions should be taken into consideration. An elderly patient with high risk MDS may still be best suited by a hypomethylating agent and/or considered for a reduced intensity allogeneic stem cell transplant.
More Information
Interpretation: IPSS-R Risk group Median survival, years Median time to 25% AML evolution* ≤1.5 Very low 8.8 Not reached >1.5 to 3 Low 5.3 10.8 >3 to 4.5 Intermediate 3 3.2 >4.5 to 6 High 1.6 1.4 >6 Very high 0.8 0.73 * Note : Patients who died were censored in the AML evolution analysis, so median time to 25% AML evolution may exceed median survival. Lower risk patients are more likely to die from another comorbidity than from AML. Figure: Kaplan-Meier curve for IPSS-R by risk group, adapted from Greenberg 2012 . Original IPSS risk groups, for historical comparison: Original IPSS Risk group Median survival 0 Low 5.7 years 0.5–1 Intermediate 1 3.5 years 1.5–2 Intermediate 2 1.2 years 2.5–3.5 High 0.4 years