Chronic Lymphocytic Leukaemia
Chronic lymphocytic leukaemia is a type of blood cancer that develops slowly, often without symptoms for months or years. While currently there is no cure, modern treatments can control the disease and help people live for many years with a good quality of life.
Table of contents
- What is chronic lymphocytic leukaemia?
- Causes and risk factors
- Symptoms
- How is CLL diagnosed?
- Treatment approaches
- Living with CLL
What is chronic lymphocytic leukaemia?
Chronic lymphocytic leukaemia (CLL) is a type of cancer that affects the blood and bone marrow, which is the spongy tissue inside bones where blood cells are made[1]. It is the most common form of leukaemia in adults[3].
In CLL, healthy white blood cells called lymphocytes mutate or change into cancerous cells. Lymphocytes are cells that help your body fight infection[2]. The term “chronic” means the cancer typically progresses slowly, and “lymphocytic” refers to the type of cells affected by the disease[2].
These abnormal cells multiply and crowd out healthy blood cells and platelets, which are cells that help blood clot[3]. The cancerous white blood cells are not able to fight infection very well. As their numbers increase in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may lead to infection, anaemia (a condition where you don’t have enough healthy red blood cells), and easy bleeding[7].
Nearly all people with CLL have what is called B-cell chronic lymphocytic leukaemia. B-cells are white blood cells that make antibodies to help fight infection[3]. There is a related condition called small lymphocytic lymphoma (SLL) that is essentially the same disease but occurs primarily in the lymph nodes rather than in the blood and bone marrow[11].
CLL affects about 5 in 100,000 people in the United States. The American Cancer Society estimates about 18,700 people will be diagnosed with CLL in 2023[3].
Causes and risk factors
Medical researchers don’t know exactly what triggers the genetic changes that cause CLL. The disease happens when certain chromosomes and genes mutate or change during your lifetime[3].
Several factors may increase your risk of developing CLL:
- Age: CLL typically affects people aged 65 and older. On average, people are 71 years old when they’re diagnosed. It can affect people starting at age 30, but it rarely occurs in children[3][7].
- Family history: Studies show people with close biological relatives who have CLL, such as a parent, sibling or child, are two to four times more likely to develop the disease[3].
- Sex: CLL is more common in males than females[3][8].
- Race: CLL is more common in white people than in people from other racial or ethnic groups[8].
- Exposure to Agent Orange: Studies show links between CLL and Agent Orange, a chemical used during the Vietnam War. The Veterans Affairs recognizes CLL as having a relationship to exposure to Agent Orange or other herbicides during military service[3][4].
- Monoclonal B-cell lymphocytosis: In this condition, you have a higher-than-normal number of identical B-cells in your blood[3].
Symptoms
You can have chronic lymphocytic leukaemia without symptoms. Many people learn they have CLL after having blood tests as part of routine physical examinations[3]. It may take months to years before you notice any symptoms[2].
When symptoms do develop, they may include:
- Swollen lymph nodes in your neck, armpit, groin or stomach. These might feel like a lump under the skin and are usually painless[2][7].
- Fatigue or feeling very tired. CLL affects your red blood cells, causing anaemia. Fatigue is a common symptom of anaemia[3].
- Fever, which is a sign of infection. CLL affects healthy white blood cells, increasing your risk of infections[3][7].
- Night sweats, sometimes drenching[2][7].
- Weight loss without trying[2][7].
- Loss of appetite[2].
- Pain or fullness in the upper left part of the belly or under your ribs. This may be caused by an enlarged spleen or liver. Cancerous white blood cells in your liver or spleen can make those organs grow[2][3].
- Frequent infections that don’t get better[2][5].
- Easy bruising or bleeding[7][8].
- Small, flat, pinpoint dark-red spots under the skin caused by bleeding, called petechiae[7][8].
How is CLL diagnosed?
If your doctor suspects you may have CLL, they will start with a physical exam. The exam checks for swollen lymph nodes in the neck, underarms and groin, and an enlarged spleen[10].
Several tests are used to diagnose CLL:
Blood tests are the main way to diagnose CLL. A complete blood count may be used to count the number of lymphocytes in a blood sample. A high number of B cells could mean chronic lymphocytic leukaemia[10]. In CLL, lymphocyte counts in the blood are usually greater than or equal to 5,000 per cubic millimetre[14].
A blood test called a peripheral blood smear may be used to see what cells in the blood look like. In chronic lymphocytic leukaemia, the smear may show a lot of small, round lymphocytes, sometimes called smudge cells[10].
Flow cytometry tests identify proteins on the surface of the cancer cells. CLL cells have certain proteins on their surfaces that help identify them. These proteins are known as markers and can also help predict how aggressive the cells are. CLL typically shows cells that are both CD5 and CD23 positive[10][14].
Genetic tests look for changes in the cancer cell DNA. Lab tests can show which DNA changes are present in the leukaemia cells. Common tests include fluorescence in situ hybridization (FISH) and tests for TP53 and IGH variants[10][14].
Bone marrow biopsy and aspiration involve collecting cells from the bone marrow. The cells are sent for testing. A healthcare professional may recommend this test if blood counts are low and there isn’t a clear reason for the low counts, though it is usually not required[10][14].
Other tests may include imaging tests like computed tomography (CT) scans to check for enlarged lymph nodes inside the body, though this is usually not required in the absence of peripheral swelling[14].
Treatment approaches
Currently, healthcare providers don’t have treatments to cure chronic lymphocytic leukaemia. However, they do have treatments to put the condition into remission, meaning you don’t have symptoms and signs of CLL. These treatments are helping people with CLL live longer[3].
It’s not possible to cure CLL, but treatment can control it[15]. Many people live a long time with the disease[12].
Watchful waiting
You may not need to have treatment straight away when you’re diagnosed with chronic lymphocytic leukaemia[15]. Research tells us that treating CLL before there are certain signs of disease progression doesn’t help people live longer. Because of this and because treatments can cause side effects, many people who are diagnosed with CLL start with active surveillance, also called watchful waiting[12].
During active surveillance, your healthcare team uses tests and exams to check if CLL is progressing or your condition is getting worse[12]. You’ll usually be seen by a doctor or nurse regularly. How often you’re seen depends on the stage of your cancer, if you’re having active treatment and how you’re feeling[15].
Active treatment options
Active treatment for CLL usually starts when symptoms develop or the disease shows signs of progression. The main treatments include:
Targeted therapy uses drugs to target specific molecules on cancer cells. These medicines aim to stop the cancer growing or help your immune system fight the cancer. You may have them on their own or with chemotherapy[12][15].
Chemotherapy uses medicines to kill cancer cells. You usually have chemotherapy with targeted cancer medicines given at the same time, called chemoimmunotherapy[12][15].
Immunotherapy helps your immune system fight the cancer. This may include treatments being studied in clinical trials such as CAR T-cell therapy and bispecific T-cell engagers[2][12].
Bone marrow transplant, also known as stem cell transplant, can sometimes be used[2][12].
Radiation therapy uses high-energy rays of radiation to kill cancer cells. You may have radiotherapy for CLL if your symptoms get worse or other treatments are not working as well anymore[15].
Surgery to remove your spleen may be needed in rare cases if you have CLL. The spleen is a small organ that filters your blood and is part of your immune system, but you can live without it[15].
Supportive treatments
You may also need treatment to prevent or control symptoms caused by CLL. This may include:
- Medicines to prevent infections
- Vaccines to prevent illness, such as the flu, pneumonia and coronavirus (COVID-19) vaccines
- Blood or platelet transfusions
- Steroid medicines[15]
What happens when symptoms come back
When your symptoms come back, this is called a relapse. This may happen slowly and you may not need to have treatment straight away. You may have several relapses while you live with CLL[15].
When your symptoms are under control, this is called remission. This can last for years[15]. You may need more treatment to control the cancer during a relapse. You’ll have more tests so doctors can work out which treatments will work best for you[15].
Living with CLL
Living with chronic lymphocytic leukaemia can be challenging, but there are ways to maintain a good quality of life through lifestyle changes and basic precautions[21].
Staying physically active
Research has shown that exercise and physical activity can improve fatigue, quality of life, and immune system function. Staying active is considered by many health care professionals to be an important component of cancer care[21].
A brisk walk or bike ride can provide moderate aerobic exercise. The American Cancer Society recommends 150 to 300 minutes of moderate exercise each week, but you can slowly work your way up to 30 minutes or more each day. Remember, any exercise is better than no exercise at all[21].
Eating a balanced diet
Eating a well-balanced diet can support your overall health. A nutritious diet and healthy weight can also improve your strength during cancer treatment and help you better tolerate side effects. Research shows that a nutritious diet can help improve immune function[21].
The Leukemia & Lymphoma Society says that the best diet for CLL includes plenty of fruits and vegetables, low-fat protein and dairy, whole grains, and olive oil or other healthy oils. Avoid foods with trans fats or added sugar, and try not to eat more than 2,300 milligrams of sodium (salt) each day[21].
Preventing infections
Because CLL affects your immune system and increases your risk of infections, it’s important to take precautions. This includes staying up to date with vaccinations, practicing good hygiene, and avoiding contact with people who are sick when possible[16].
Emotional support
Coping with a long-term condition like CLL can be difficult. It’s normal to feel anxious, especially knowing that symptoms can come back at any time. Help and support are available[19].
Talking to your friends and relatives about your cancer can help and support you. You might also find it easier to talk to someone other than your own friends and family, such as a counsellor or support group[19].
Taking in information can be difficult, especially when you have just been diagnosed. Make a list of questions before you see your doctor. Take someone with you to remind you what you want to ask. They can also help you to remember the information that was given[19].
Managing daily life
Having cancer may require you to make some changes to your routine, but it doesn’t have to change everything about your life. Taking time for yourself to relax and de-stress is a valuable part of your recovery[16].
Practical tips for managing daily life include making lists to help you remember things and questions for your team, keeping a calendar with all appointments, setting goals, and planning enjoyable activities[19].
It can be difficult living with CLL and knowing it can come back at any time. It can help to get support from family, friends or a support organisation if you get anxious between appointments[15].
