IPSS-R

IPSS-R in Myelodysplastic Syndromes (MDS)

IPSS-R = Revised International Prognostic Scoring System

IPSS-R = refined prognostic tool in MDS using cytogenetics, blasts, Hb, platelets, and ANC. It stratifies patients into 5 risk groups, guiding prognosis and therapy (supportive vs disease-modifying)

What It Measures

IPSS-R uses five disease features at diagnosis:

1. Cytogenetics (karyotype)
   • Stratified into very good, good, intermediate, poor, very poor
   • Example: del(5q) = good, complex karyotype (≥3 abnormalities) = very poor
2. Bone marrow blast %
   • <2%
   • 2–<5%
   • 5–10%
   • 10%
3. Hemoglobin (g/dL)
4. Platelet count (×10⁹/L)
5. Absolute neutrophil count (ANC ×10⁹/L)

Each category assigns points → summed for a total score.

Risk Categories

Total score stratifies patients into five risk groups:

• Very Low
• Low
• Intermediate
• High
• Very High

Each corresponds to median survival and risk of progression to AML.

Clinical Use

• Prognosis: Predicts overall survival and risk of AML transformation.
• Treatment guidance:
  • Lower risk (Very Low, Low, some Intermediate):
    • Supportive care (transfusions, growth factors)
    • Lenalidomide (esp. del(5q))
    • Erythropoiesis-stimulating agents (ESAs)
    • Luspatercept for anemia with ring sideroblasts
  • Higher risk (High, Very High, some Intermediate):
    • Hypomethylating agents (azacitidine, decitabine)
    • Allogeneic stem cell transplant (curative option)
    • Clinical trials