International Prognostic Index for Chronic Lymphocytic Leukemia (CLL-IPI)

CLL-IPI Calculator
Age >65
Clinical Stage (Binet B/C or Rai I-IV)
β2-Microglobulin >3.5 mg/L
Unmutated IGHV
del(17p) and/or TP53 Mutation
CLL-IPI Score: 0
Low Risk
CLL-IPI 0: Low risk. 5-year survival ~93%. Observation recommended.
Stratifies patients with chronic lymphocytic leukemia into four risk categories.

Why Use

Many patients with CLL will have an indolent course and not require treatment for many years, while others will have a shorter time to first treatment. The CLL-IPI combines clinical, laboratory and genetic risk factors into a single score that can be used to help estimate time to first treatment as well as overall survival.

When to Use

Patients with new diagnosis of chronic lymphocytic leukemia (CLL), to estimate prognosis and time to first treatment.

Formula

Addition of the selected criteria: Criteria Selection Points Age ≤65 years 0 >65 years 1 Clinical stage Binet A or Rai 0 0 Binet B-C or Rai I-IV 1 Serum β2 microglobulin, mg/L (or µg/mL) ≤3.5 0 >3.5 2 IGHV mutational status Mutated 0 Unmutated 2 TP53 status No abnormalities 0 Deletion 17p (FISH) and/or TP53 mutation (sequencing) 4

Pearls / Pitfalls

Developed using patient data from before use of targeted agents such as ibrutinib and venetoclax, which are known to have greater efficacy in patients with TP53 alterations. While treatment type was not an independent factor in the CLL-IPI, TP53 status was, and thus the use of novel agents may have an effect not currently measured in the CLL-IPI.

Management

Guidelines have not yet incorporated the CLL-IPI scoring system into management algorithms. Risk categories should be used to inform prognosis, and closer monitoring for higher risk patients should be considered. Notably, survival estimates were based on assessments from before the era of targeted therapies for CLL, and this should be taken into consideration when counseling patients.

Advice

The CLL-IPI categorizes patients into four risk groups from low risk to very high risk. The decision to treat should not be based on the risk score, since it has not been evaluated for that purpose. Indications for treatment remain the same, but higher risk patients may warrant closer initial monitoring.

More Information

Interpretation: CLL-IPI Score Risk 5-year survival 0-1 Low risk 93.2% 2-3 Intermediate risk 79.3% 4-6 High risk 63.3% 7-10 Very high risk 23.3%

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