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Doctors use staging to help them predict chronic lymphocytic leukemia's (CLL's) progression and develop an appropriate treatment plan. Doctors use one of two staging systems: the Rai staging system or the Binet staging system.

Staging systems consider certain factors such as:

  • the elevation of your blood and marrow leukemic lymphocyte counts
  • your lymph nodes' size and distribution
  • your spleen's size
  • the extent of your decreased blood platelet counts
  • the degree of anemia

Rai Staging System

The Rai staging system classifies CLL into the following five stages based on factors at the time of diagnosis:

Low-Risk 0
  • Abnormal increase in the number of lymphocytes in the blood and marrow
Intermediate
Risk I
  • Abnormal increase in the number of lymphocytes in the blood and marrow
  • Enlarged (swollen) lymph nodes
Intermediate
Risk II
  • Abnormal increase in the number of lymphocytes in the blood and marrow
  • Enlarged (swollen) lymph nodes, liver or spleen
High Risk III
  • Abnormal increase in the number of lymphocytes in the blood and marrow
  • Anemia (low red-cell count)
High Risk IV
  • Abnormal increase in the number of lymphocytes in the blood and marrow
  • Low platelet count

Binet Staging System

The Binet staging system is less commonly used. It classifies CLL by three stages: A, B and C.

A Stage
  • Abnormal increase in the number of lymphocytes in the blood
  • Less than three swollen lymph nodes that can be felt under the skin
B Stage
  • Abnormal increase in the number of lymphocytes in the blood
  • Three or more swollen lymph nodes that can be felt under the skin
C Stage
  • Abnormal increase in the number of lymphocytes in the blood
  • Three or more swollen lymph nodes that can be felt under the skin
  • Anemia (low red cell count) or low platelet count

Other Test Results

When your hematopathologist examines your blood cells to decide staging, he or she may find other factors that can affect your CLL prognosis. The following can be signs of a faster-growing disease (higher-risk CLL):

  • Blood lymphocyte doubling time. If your lymphocyte count doubles in one year, you have higher-risk CLL and need closer follow-up care. A lymphocyte number that remains stable shows a relatively lower risk.
  • Beta 2-microglobulin (B2M). B2M is protein that's shed from CLL cells. A higher level of B2M may mean a greater extent of the disease.
  • CD38. A characteristic marking (antigen) called CD38 (CD stands for "cluster designation") on the cells' surface can indicate higher-risk CLL.
  • Unmutated IgHv. The presence of a marker on the CLL cells, called unmutated immunoglobulin heavy chain variable region gene (IgHv), suggests higher-risk disease.
  • ZAP 70 (Zeta-associated protein 70). High levels of this cell protein suggest higher-risk disease. It should be noted that further study in clinical trials is needed to standardize the assessment of ZAP-70. The National Comprehensive Cancer Network (NCCN) guidelines state that the evaluation of ZAP-70 expression by flow cytometry can be challenging and is not recommended outside of a clinical trial.
last updated on Friday, May 11, 2012
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