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Life can look very different depending on whether you’re living with stage 0 or stage 4 of chronic lymphocytic leukemia (CLL). Knowing more about the stages of CLL can help you understand what life with this disease might look like if it progresses and what symptoms you might expect.
When you’re first diagnosed with CLL, 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 your overall outlook.
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 doesn’t usually form tumors. Instead, the leukemia cells develop from blood-forming cells in the bone marrow. Leukemia 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, oncologists (doctors who specialize in cancer) can use two staging systems. The Rai system is commonly used in the United States, and it classifies CLL into five numbered stages, stages 0 to 4. 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.
The Rai staging system categorizes CLL 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, a situation known as lymphocytosis.
The Rai staging system divides CLL into five stages, starting with zero. Doctors stage leukemia based on many factors, including the presence of:
The stages can also be broken down into three risk groups:
You may experience different symptoms of CLL depending on the stage of blood cancer you’re living with.
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 as an incidental finding (something doctors discover without looking for it) on routine blood tests. Symptoms, if there are any, 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.”
In stage 1 CLL, there is lymphocytosis plus enlarged lymph nodes. The liver and spleen are still normal in size, and blood test results show that red blood cell and platelet counts are near normal. Stage 1 is considered intermediate risk.
Symptoms are still minimal at stage 1. 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.”
In stage 2 CLL, there is lymphocytosis, and the spleen or liver is enlarged. The 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. Like stage 1, stage 2 is intermediate risk.
Some members of MyLeukemiaTeam report noticing organ enlargement at this stage. One said, “I had some spleen enlargement noted at my last visit.”
In stage 3 CLL, there is lymphocytosis, and the liver, spleen, or lymph nodes may be enlarged. Blood test results show that hemoglobin is low, indicating anemia, and platelet counts are near normal. Stage 3 is considered high risk.
In stage 3, you might develop symptoms of anemia, which can worsen fatigue.
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 are getting me down.”
You may need treatments to help manage anemia. Your doctor may recommend supplements or blood transfusions to manage symptoms of anemia, but treatment will depend on the specific cause of the anemia.
In stage 4 CLL, there is lymphocytosis, and the liver, spleen, or lymph nodes are likely enlarged. Blood test results show that platelet counts are low. Red blood cell counts may also be low. Stage 4 is high risk.
At this point, you’re likely to experience symptoms of anemia. If your organs become swollen enough, you may feel overly full after only a few bites of food. “My tummy is swollen because of my spleen,” one MyLeukemiaTeam member shared.
Due to the low platelet count, you may notice symptoms like:
There’s no way to predict when you’ll notice symptoms of CLL, because each person 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 be worrying.
One member shared, “I’ve noticed some weight loss. Needless to say, I’m scared.”
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.
Today, doctors pay close attention to genetic mutations (changes) in leukemia cells, such as TP53 changes and immunoglobulin heavy chain variable region (IGHV) status. These two lab results can tell them a lot about how the CLL may behave and which treatments are likely to work best.
Depending on the specific characteristics of your leukemia, existing CLL treatments or clinical trials might provide the most promising options. Many of these details are assessed during laboratory examinations of your leukemia cells.
Older age — 70 and up — tends to worsen prognosis. Other factors that might lead to a worse prognosis include:
Factors that might improve your prognosis include:
Factors such as ZAP70, CD38, and IGHV mutations can help doctors predict whether CLL will grow slowly or quickly. Slow-growing CLL generally has a better prognosis, and people tend to live longer.
Many people with early-stage or slow-growing CLL can safely delay treatment and stay on “watchful waiting,” with regular monitoring.
Starting CLL treatment early doesn’t usually improve your prognosis. But if symptoms become an issue or CLL symptoms progress, your doctor may advise starting therapy.
Most people begin with targeted tablets — such as Bruton’s tyrosine kinase (BTK) or BCL-2 inhibitor-based treatments — which are often very effective. Older chemotherapy is used far less often now, and it’s usually reserved for specific situations.
If you choose to start treatment, your options may depend partly on details about your cancer cells and their genetic mutations. Treatment options may include:
When to start treatment — and which type — should be a shared decision you make in partnership with your doctor.
Read about eight ways to live better with CLL.
On MyLeukemiaTeam, people share their experiences with leukemia, get advice, and find support from others who understand.
How have your symptoms changed as CLL has progressed to more advanced stages? Let others know in the comments below.
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What Stage CLL Would I Have If I Have Lymphocytosis (around 4000), No Swollen Lymph Nodes, Liver Or Spleen. But I Am Mildly Anemic?
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I asked my oncologist which stage I am in. He said stage 2. I am on Calquence and responding well. Hope it keeps up!
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