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    Patent Pending

    Revised International Prognostic Scoring System (IPSS-R) for Myelodysplastic Syndrome (MDS)

    Stages patients with MDS.
    Favorite

    INSTRUCTIONS

    Use at the time of diagnosis, before starting treatment.

    When to Use
    Pearls/Pitfalls
    Why Use

    Patients newly-diagnosed with myelodysplastic syndrome.

    • 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.

    Can help determine whether to treat or observe, and what type of treatment, based on risk score.

    Very good: del(11q) or -Y
    0
    Good: normal karyotype, del(20q), del(5q), del(12p), or double including del(5q)
    +1
    Intermediate: +8, del(7q), i(17q), +19, or any other single or double independent clone
    +2
    Poor: -7, inv(3)/t(3q)/del(3q), double including -7/del(7q), or complex (3 abnormalities)
    +3
    Very poor: complex >3 abnormalities
    +4
    ≤2
    0
    >2 to <5
    +1
    5 to 10
    +2
    >10
    +3
    ≥10 (≥100)
    0
    8 to <10 (80 to <100)
    +1
    <8 (<80)
    +1.5
    ≥100
    0
    50 to <100
    +0.5
    <50
    +1
    ≥0.8
    0
    <0.8
    +0.5

    Result:

    Please fill out required fields.

    Next Steps
    Evidence
    Creator Insights

    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.

    Formula

    Addition of the selected points:

       

    Points

    Cytogenetic group

    Very good: del(11q) or -Y

    0

    Good: normal karyotype, del(20q), del(5q), del(12p), or double including del(5q)

    1

    Intermediate: +8, del(7q), i(17q), +19, or any other single or double independent clone

    2

    Poor: -7, inv(3)/t(3q)/del(3q), double including -7/del(7q), or complex (3 abnormalities)

    3

    Very poor: complex >3 abnormalities

    4

    Medullary blasts, %

    ≤2

    0

    >2 to <5

    1

    5 to 10

    2

    >10

    3

    Hemoglobin, g/dL (g/L)

    ≥10 (≥100)

    0

    8 to <10 (80 to <100)

    1

    <8 (<80)

    1.5

    Platelets, ×103/µL or 109/L

    ≥100

    0

    50 to <100

    0.5

    <50

    1

    ANC, ×103/µL or 109/L

    ≥0.8

    0

    <0.8

    0.5

     

    Original IPSS, for historical comparison:

       

    Points

    Bone marrow blasts, %

    <5

    0

    5–10

    0.5

    11–20

    1.5

    21–30

    2

    Karyotype

    Good: normal, −Y, del(5q), or del(20q)

    0

    Intermediate: other abnormalities not listed under good or poor

    0.5

    Poor: complex (≥3 abnormalities) or chromosome 7 anomalies

    1

    Number of cytopenias

    0–1

    0

    2–3

    0.5

    Facts & Figures

    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, 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

     

    Evidence Appraisal

    The original IPSS analyzed data from 816 untreated MDS patients by multivariate analysis to devise a score that weighted certain cytogenetic abnormalities, percentage of bone marrow blasts and number of cytopenias based on their correlation with outcome. The revised IPSS (IPSS-R) was developed in 7,012 patients and added some additional cytogenetic abnormalities and used specific blood count value ranges in the calculation. The IPSS had four categories, while the IPSS-R has better discrimination with five categories. Both scores have been validated; however, the IPSS-R is more frequently used and is more precise. The validation for the IPSS-R was performed on 200 external patients and outperformed the IPSS.  

    Literature

    Dr. Peter Greenberg

    About the Creator

    Peter Greenberg, MD, is professor emeritus of hematology at Stanford University. He is also director of the Stanford Myelodysplastic Syndrome (MDS) Center, coordinator of the International Working Group for Prognosis in MDS and chair of the National Comprehensive Cancer Network MDS Practice Guidelines Panel. Dr. Greenberg’s research focuses on evaluating molecular and genetic abnormalities in myelodysplastic syndromes.

    To view Dr. Peter Greenberg's publications, visit PubMed

    Content Contributors
    Reviewed By
    • Eytan Stein, MD
    About the Creator
    Dr. Peter Greenberg
    Content Contributors
    Reviewed By
    • Eytan Stein, MD