Cancer stages provide a quick way for doctors to talk about how much cancer has spread or grown. Staging helps doctors determine a person’s outlook and develop the right treatment plan.
Most types of cancer are assigned stages based on the size of tumor growth. Lower stages indicate smaller tumors, while higher stages indicate that tumors have grown and spread.
Chronic lymphocytic leukemia (CLL), like all types of leukemia, is a cancer of the blood. It doesn’t cause tumors to form, but CLL can still be staged. Staging is done by looking at the ratio of cancerous white blood cells to healthy blood cells.
There are two different staging systems used for CLL, the Rai symptom and the Binet system.
In the United States, the Rai system is used more often. This system bases classes on the presence of lymphocytosis, an abnormally high number of lymphocytes in blood and bone marrow.
The system also considers a person’s overall blood count and any enlargement of their lymph nodes, spleen, or liver.
There are five Rai system CLL stages:
|Stage 0||Lymphocytosis, but no enlargement of the lymph nodes, spleen, or liver. Red blood cell and platelet counts will be normal or close to normal at this stage.|
|Stage 1||Lymphocytosis and enlargement of the lymph nodes. No enlargement of spleen or liver. Red blood cell and platelet counts will be normal or close to normal at this stage.|
|Stage 2||Lymphocytosis and enlargement of the spleen. Enlargement of the liver is possible but no enlargement of lymph nodes. Red blood cell and platelet counts will be normal or close to normal at this stage.|
|Stage 3||Lymphocytosis and a low red blood cell count. Enlargement of the spleen, liver, and lymph nodes is possible. Platelet counts will be normal or close to normal.|
|Stage 4||Lymphocytosis and a low platelet count. Enlargement of the lymph nodes, spleen, and liver is possible. Red blood cell count might low or normal.|
The Binet system is commonly used in European countries. The Binet system bases classes on the number of enlarged lymphoid tissue groups.
Lymphoid tissue is found in the lymph nodes in your neck, groin, and underarms, as well as in your spleen and liver. The system also looks at your total blood cell count.
There are three Binet system CLL stages:
|Stage A||Less than three lymphoid tissue areas are enlarged. Red blood cell and platelet counts are normal.|
|Stage B||Three or more lymphoid tissue areas are enlarged. Red blood cell and platelet counts are normal.|
|Stage C||Any number of lymphoid tissue areas are enlarged. Blood cell counts find too few red blood cells, too few platelets, or both.|
Lower stages of either system have a better outlook than higher stages. Higher stages indicate that the cancer has spread in your body. This can make treatment more challenging.
The Rai system is separated into three risk groups. These risk groups represent the survival rates and overall outlook of the stages. The risk groups in the Rai system are:
- Low risk: Stage 0 is considered low risk.
- Intermediate risk: Stages 1 and 2 are considered intermediate risk.
- High risk: Stages 3 and 4 are considered high risk.
The Binet system is sometimes used along with other factors such as age, genetic markers, and cellular markers to determine outlook. Your risk stage is determined by a combination of these factors.
This system is called the Chronic Lymphocytic Leukaemia International Prognostic Index. In general, stage A isn’t considered to be a factor that could raise your risk. Stages B and C do increase your risk but don’t automatically move you to a different risk group.
There are factors other than staging that impact your CLL outlook. Your doctor will assess all of these factors to determine the best treatment plan for you.
General factors that impact outlook include:
- speed of lymphocyte growth
- amount of normal bone marrow cells replaced by leukemia cells
- blood levels of beta-2 microglobulin
- the presence of chromosomal changes in cancer cells
- the ability of cancer cells to produce antibodies
Factors that positively impact your outlook include:
- being younger than 65
- having slow-growing cancer cells
- having a limited number of affected bone marrow cells
- having a low number of cells with the ZAP-70 or CD38 gene
- having cells with a changed IGHV gene present
- having part of chromosome 13 deletion
Factors that negatively impact your outlook include:
- being older than 65
- having the total number of cancer cells double in less than a year
- having a large number of affected bone marrow cells
- having high blood levels of beta-2 microglobulin
- having a high number of cells with the ZAP-70 or CD38 gene
- having trisomy 12 in cancer cells
- having parts of chromosomes 17 or 11 deleted
- not having cells with the changed IGHV genes present
- not having cells that contain the TP53 gene
There’s no cure for CLL, but many people are able to live long and fulfilling lives after a CLL diagnosis.
Researchers are always working to develop new treatments for CLL. These treatments will likely increase the survival rate in the years to come. Additionally, your specific staging and prognostic factors make a big difference.
Talk with your doctor and ask questions if you’re not sure about your stage or outlook.
It’s important to have support no matter what stage your CLL is in. A social worker, psychologist, or counselor can be a great place to start. They can offer you a way to work through the complex emotions a cancer diagnosis can trigger.
Support groups are another great way to feel connected during your CLL treatment. You can look into local groups at your hospital or cancer center, or look into online groups.
The Leukemia & Lymphoma Society also maintains a long list of support resources for people managing CLL and other types of leukemia.
CLL staging is a quick way for doctors to discuss how far cancer has progressed and what the treatments are.
The United States primarily uses the five-stage Rai system. The Rai system can be broken down into low, intermediate, and high-risk groups.
The European Binet system has three stages. Both symptoms assign higher stages to CLL that have spread throughout the body and will be more difficult to treat.
The overall survival rates for CLL are encouraging and will likely continue to increase as new cancer treatments are developed.