Chronic Lymphocytic Leukemia: Staging

All forms of non-Hodgkin lymphoma, including chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL), are divided into four stages, based on how far the disease has spread.

Stages I and II are considered localized, while stages III and IV are considered advanced, widespread or disseminated. Knowing the stage of a person's lymphoma helps their healthcare team to determine the most appropriative and effective treatment plan for them.

How Is CLL Staged?

Staging is used to describe how widely the cancer has spread in patients with CLL and other types of cancer. Because of how blood and bone marrow are involved in CLL, these staging systems are different from those used for other types of NHL, including SLL. There is no single standardized staging system for CLL. Patients with CLL are staged using either the Rai staging system or the Binet classification system. Doctors in the United States tend to use the Rai system, while the Binet system is more popular in Europe.

The following table shows the Rai staging system.

Stage Description Risk Group
0

The blood and bone marrow show an abnormal
increase in the number of lymphocytes

This condition is called lymphocytosis

CLL at this stage is slow growing (indolent)

Low
I Lymphocytosis with enlarged nodes Intermediate
II Lymphocytosis, enlarged nodes, and an enlarged spleen (splenomegaly) and/or an enlarged liver (hepatomegaly) Intermediate
III Lymphocytosis, enlarged nodes, enlarged spleen and/or liver, and anemia (hemoglobin less than 11 grams per deciliter) High
IV Lymphocytosis, enlarged nodes, enlarged spleen and/or liver, anemia, and low platelet count (thrombocytopenia: less than 100,000 per microliter) High

 

The table below shows the Binet classification system.

Stage Description Match-Up With Rai Stages
A Less than three enlarged areas (lymph nodes or spleen) without low hemoglobin (anemia) and low platelet count (thrombocytopenia) Rai stages 0, I, and II
B Three or more areas of lymph node involvement without anemia and thrombocytopenia Rai stages I and II
C Anemia and/or thrombocytopenia, along with any number of enlarged areas Rai stages III and IV

 

How Is SLL Staged?

Staging is used to describe how widely the cancer has spread in patients with NHL. The Ann Arbor staging system has been used for staging NHLs other than CLL. Although the older staging system is still in use, a modification of the Ann Arbor staging system—the Lugano Classification—was proposed in 2014, which is shown in the following figure. There are two main classifications (limited and advanced disease) and four stages of lymphoma designated by the Roman numerals I through IV. Stages I and II are considered limited disease, although Stage II can be considered advanced in some cases. Stages III and IV are considered advanced disease.

Staging of NHL (Lugano Classification)

  • Stage I: Single lymph node or group of adjacent nodes
  • Stage II: Two or more groups of lymph nodes on the same side of the diaphragm
  • Stage III: Lymph nodes on both sides of the diaphragm
    • Lymph nodes above the diaphragm with spleen involvement
  • Stage IV: Widespread disease in lymph nodes and organ involvement

Stage II disease that is also called bulky, meaning that the patient has a tumor greater than 10 centimeters (4 inches) wide depending on the type of NHL, can sometimes be considered advanced disease.

The newer staging system is similar to the previous Ann Arbor staging system, except that the "A" and "B" designations are no longer used except for the staging of Hodgkin lymphoma (HL).

Doctors use the stage of disease, test results, and/or other factors to help decide the best time to begin treatment and what treatments are likely to be the most effective for each patient.

Being diagnosed with advanced NHL is common in most patients. Keep in mind that these advanced stages can be successfully treated.