Doctors use staging to help them predict chronic lymphocytic leukemia's (CLL's) progression and develop an appropriate treatment plan. Two staging systems, the Rai system and the Binet System, have been used throughout the world. In 2016, a new prognostic model called the CLL International Prognostic Index (CLL-IPI) was released, enabling a more targeted management of CLL.
Staging systems for CLL take into account:
- Abnormal increase in number of lymphocytes (lymphocytosis)
- Presence of enlarged lymph nodes
- Presence of enlarged spleen and/or liver
- Presence of anemia (abnormal decrease in the number of red blood cells)
- Presence of thrombocytopenia (abnormal decrease in the number of platelets)
Rai Staging System
The Rai staging system classifies CLL into three separate risk groups.
| Stage | Characteristics |
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Low Risk |
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Intermediate Risk |
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High Risk |
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Binet Staging System
The Binet staging system classifies CLL into three stages.
| Stage | Characteristics |
| A Stage |
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| B Stage |
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| C Stage |
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CLL International Prognostic Index (CLL-IPI)
The CLL-IPI combines genetic, biochemical and clinical parameters to categorize patients into four prognostic risk groups. Five independent prognostic factors were identified:
- TP53 deleted or mutated = 4 points
- Unmutated IGHV = 2 points
- Serum beta-2 microglobulin concentration > 3.5 mg/L = 2 points
- Rai Stage I - V or Binet Stage B - C = 1 point
- Patient age > 65 years = 1 point
The CLL-IPI provides treatment recommendations by risk roup based on the points system.
| CLL-IPI Category | Risk Score | Treatment Recommendations |
| Low Risk | 0-1 | Do not treat |
| Intermediate Risk | 2-3 | Do not treat unless the disease is highly symptomatic |
| High Risk | 4-6 | Treat unless the patient is asymptomatic |
| Very High Risk | 7-10 | If the decision is made to treat, use novel agents or treatment in a clinical trial rather than chemotherapy |
Related Links
- Diagnosis
- Download or order The Leukemia & Lymphoma Society's free booklet, Chronic Lymphocytic Leukemia.