CLL Staging

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

Low Risk 
(Stage 0)
  • Abnormal increase in the number of lymphocytes in the blood and marrow

Intermediate Risk
(Stages I & II)
  • Abnormal increase in the number of lymphocytes in the circulating blood and the marrow
  • Enlarged lymph nodes
    OR
  • Abnormal increase in the number of lymphocytes in the circulating blood and the marrow
  • Enlarged spleen and/or liver

High Risk
(Stages III & IV)
  • Abnormal increase in the number of lymphocytes in the circulating blood and the marrow
  • Anemia (hemoglobin <11g/dL)
    OR
  • Abnormal increase in the number of lymphocytes in the circulating blood and the marrow
  • Thrombocytopenia (platelet counts <100,000/uL)

 

Binet Staging System

The Binet staging system classifies CLL into three stages. 

Stage Characteristics
A Stage
  • No anemia (hemoglobin ≥10g/dL)
  • No thrombocytopenia (platelets ≥100,000/mm3 )
  • Less than 3 areas of lymphoid tissue enlargement
B Stage
  • No anemia (hemoglobin ≥10g/dL)
  • No thrombocytopenia (platelets ≥100,000/mm3 )
  • 3 or more areas of lymphoid tissue enlargement
C Stage
  • Anemia (hemoglobin <10g/dL)
  • Thrombocytopenia (platelets <100,000/mm3 )
  • Any number of areas of lymphoid tissue enlargement

 

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 group 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

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