When you’re first diagnosed with chronic lymphocytic leukemia, your doctor will run several tests to determine the stage of your cancer. Knowing the stage helps determine what kind of treatment you will receive and the outlook for your diagnosis.

Staging Chronic Lymphocytic Leukemia

Most cancers are staged by the size of the tumor and how much the cancer has spread throughout the body. However, CLL is a blood cancer and does not usually form tumors. Instead, the leukemia cells can be found in the blood-forming cells of the bone marrow. It can also spread to the liver, lymph nodes, and spleen.

Staging systems are standardized ways for doctors to describe cancer and make a diagnosis. When diagnosing CLL, oncology doctors can use two staging systems. The Rai system is commonly used in the United States. The Binet staging system is commonly used in Europe and classifies CLL into stage A, stage B, and stage C. This article focuses on the Rai system.

Rai Staging System

The Rai staging system categorizes CLL into five stages based on high white blood cell counts and other factors. Lymphocytes are one type of white blood cell and are an infection-fighting part of the immune system. In some types of leukemia and lymphoma, the body makes an abnormally high number of lymphocytes, which is called lymphocytosis.

The Rai staging system divides CLL into five stages, starting with zero, based on many factors. These include the presence of:

• Lymphocytosis
• Enlarged liver
• Enlarged spleen
• Enlarged lymph nodes
• Decreased number of platelets
• Anemia (low hemoglobin, the iron-containing part of red blood cells)

The stages can also be broken down into three risk groups:

• Low risk (stage 0)
• Intermediate risk (stages 1 and 2
• High risk (stages 3 and 4)

Living With Different Stages of Chronic Lymphocytic Leukemia

You may experience different symptoms depending on the stage of CLL you’re living with.

Stage 0

In stage 0 CLL, there is lymphocytosis but no enlargement of the liver, spleen, or lymph nodes. Blood test results show that red blood cell and platelet counts are normal. Stage 0 is low risk.

People in stage 0 may have no symptoms of leukemia. Lymphocytosis is usually detected on routine blood tests. Any symptoms experienced are very few and usually don’t interfere with daily life. One MyLeukemiaTeam member with stage 0 CLL explained, “The only symptom I have so far is that I tire a little quicker than I used to.”

Stage 1

In stage 1 CLL, there is lymphocytosis plus enlarged lymph nodes. The liver and spleen are still normal size, and blood test results show that red blood cell and platelet counts are near normal. Stage 1 is intermediate risk.

Symptoms are still minimal at stage 1. You may notice enlarged lymph nodes but otherwise feel close to normal. You may experience more fatigue and begin to have night sweats.

One member of MyLeukemiaTeam who went through this stage shared, “You might get night sweats or feel a little more tired than you used to.”

Another person with stage 1 CLL added, “I’ve been taking longer naps in the afternoon.”

Stage 2

In stage 2 CLL, there is lymphocytosis, and the spleen is enlarged. The liver and lymph nodes may or may not be enlarged, and blood test results show that red blood cell and platelet counts are near normal. An enlarged spleen or liver could cause some abdominal pain. Stage 2 is also intermediate risk.

Some members of MyLeukemiaTeam have mentioned organ enlargement at this stage. One said, “I had some spleen enlargement noted at my last visit.”

Stage 3

In stage 3 CLL, there is lymphocytosis, and some combination of the liver, spleen, and lymph nodes may be enlarged. Blood test results show that hemoglobin is low, indicating anemia, and platelet counts are near normal. Stage 3 is high risk.

In stage 3, you might develop symptoms of anemia, which can make you a lot more tired than you were before. One MyLeukemiaTeam member wrote, “I have anemia along with the CLL, so I get tired easy.”

Another added, “I have anemia, and the lack of energy and chest pain is getting me down.”

You may need treatments to help manage anemia. Your doctor may recommend iron supplements, vitamin B12 supplements, or blood transfusions. Always discuss a new supplement with your doctor before you begin taking it.

Stage 4

In stage 4 CLL, there is lymphocytosis, and the liver, spleen, or lymph nodes are likely enlarged. Blood test results show that red blood cell and platelet counts are low. Stage 4 is high risk.

At this point, you’re likely to experience symptoms of anemia. If your organs get large enough, you may feel overly full. “My tummy is swollen because of my spleen,” one MyLeukemiaTeam member shared.

Due to the low platelet count, you may notice symptoms like:

• Bruising or bleeding easily
• Nosebleeds
• Bleeding longer than usual if skin is cut
• Pinkish or reddish urine

There’s no way to tell exactly when you will notice symptoms of CLL, because everybody is different. But by stage 4, you’re more likely to experience symptoms like shortness of breath, low-grade fever, and weight loss, which can become worrying. One member voiced, “I’ve noticed some weight loss. Needless to say, I’m scared.”

Predicting Prognosis of Chronic Lymphocytic Leukemia

Doctors use the CLL International Prognostic Index to help predict a person’s disease prognosis (outlook). These factors aren’t taken into consideration during traditional staging, but they can be used to determine the right treatment options.

Depending on the specific characteristics of your leukemia, existing CLL treatments or clinical trials might provide the most promising treatment. Many of these details are assessed during laboratory examinations of your leukemia cells.

Older age — 65 and up — tends to worsen prognosis. Other factors that might lead to a worse prognosis include:

• High levels of certain substances in the blood, such as beta-2 microglobulin
• A large number of CLL cells with increased amounts of the protein ZAP70 or CD38
• CLL cells with an unmutated gene for an immune-system protein called immunoglobulin heavy chain variable region (IGHV)

Factors that might improve your prognosis include:

• A small number of cells with higher amounts of ZAP70 or CD38
• CLL cells with a mutation in the IGHV gene

Factors such as ZAP70, CD38, and IGHV mutations can help doctors divide CLL into slow-growing or fast-growing cases. Slow-growing CLL generally has a better prognosis, and people tend to live longer.

Starting Treatment for Chronic Lymphocytic Leukemia

With CLL, early treatment doesn’t improve your prognosis. People with early-stage, slow-growing CLL may be able to delay treatment and go on “watchful waiting” with regular monitoring. Delaying treatment allows you to avoid potential side effects of treatment. If your symptoms become bothersome or your CLL shows signs of progressing, your doctor may advise starting treatment at that point.

If you choose treatment, your options may depend partly on details about your cancer cells and their genetic mutations. Treatment options may include:

• Traditional chemotherapy, immunotherapy, and radiation therapy
• Targeted therapy
• Immunotherapy like chimeric antigen receptor (CAR) T-cell therapy

When to start treatment — and which type — should be a shared decision you make in partnership with your doctor.

Talk With Others Who Understand

MyLeukemiaTeam is the social network for people with leukemia and their loved ones. Every day, more than 17,000 members come together to ask questions, give advice, and share their stories with others who understand life with leukemia.

Do you know what stage of chronic lymphocytic leukemia you have and what to expect moving forward? Share your experience in the comments below, or start a conversation by posting on your Activities page.