Lymphocytic Leukaemia

Lymphocytic leukaemia is a cancer affecting white blood cells in the blood and bone marrow, with chronic forms progressing slowly over time and acute forms developing rapidly.

Table of contents

• What is lymphocytic leukaemia?
• Types of lymphocytic leukaemia
• Signs and symptoms
• Causes and risk factors
• Diagnosis
• Treatment
• Living with the condition

What is lymphocytic leukaemia?

Lymphocytic leukaemia is a type of cancer that affects the blood and bone marrow, which is the spongy tissue inside bones where blood cells are made. This condition affects a specific group of white blood cells called lymphocytes, which normally help your body fight infection and disease.^[1][3]

In lymphocytic leukaemia, the bone marrow produces too many abnormal lymphocytes. These cells fail to develop properly and cannot fight infections as healthy cells do. As the number of abnormal cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This can lead to infection, anemia, and easy bleeding or bruising.^[6][10]

The abnormal lymphocytes may also spread to the lymph nodes, spleen, and liver, causing these organs to enlarge.^[4]

Types of lymphocytic leukaemia

There are two main types of lymphocytic leukaemia: chronic and acute. Each behaves differently in the body.^[1][3]

Chronic lymphocytic leukaemia (CLL) develops slowly over time. Many people with this form do not experience symptoms right away and may live for years before needing treatment. CLL is the most common type of leukaemia in adults, particularly affecting people over age 65. On average, people are diagnosed at age 70 or 71.^[1][2][4]

Acute lymphocytic leukaemia (ALL) progresses rapidly and creates immature blood cells rather than mature ones. This form is most common in children, though it can also occur in adults. Treatment for ALL in children often results in a good chance of cure, but the outlook is less favorable in adults.^[3]

Within chronic lymphocytic leukaemia, there are subtypes based on which type of white blood cell is affected. Nearly all people with CLL have B-cell chronic lymphocytic leukaemia, which affects B lymphocytes. B cells make antibodies that target viruses, bacteria, and cancer cells. A much smaller number of people develop T-cell prolymphocytic leukaemia, which affects T lymphocytes that control the body’s immune system response.^[2][4]

When cancer primarily affects the lymph nodes instead of the blood and bone marrow, it’s called small lymphocytic lymphoma (SLL). SLL and CLL are essentially the same disease but occur in different locations and are treated the same way.^[4][5]

Signs and symptoms

Many people with chronic lymphocytic leukaemia do not have symptoms when first diagnosed. The condition may be discovered during routine blood tests as part of a regular physical examination. It may take months or even years before symptoms appear.^[1][2][4]

When symptoms do develop, they may include:^[1][4][6]

• Swollen lymph nodes in the neck, armpit, groin, or stomach. These may feel like painless lumps under the skin
• Fatigue or feeling very tired
• Fever
• Frequent infections that don’t get better
• Night sweats that drench your clothes and sheets
• Weight loss without trying
• Loss of appetite
• Pain or a feeling of fullness below the ribs, which may be caused by an enlarged spleen or liver
• Easy bruising or bleeding
• Small, flat, dark-red spots under the skin caused by bleeding

In acute lymphocytic leukaemia, symptoms may also include bleeding from the gums, frequent or severe nosebleeds, bone pain, pale skin, shortness of breath, and general weakness or decreased energy.^[3]

Causes and risk factors

Lymphocytic leukaemia occurs when bone marrow cells develop changes in their genetic material or DNA. These changes, called mutations, cause the cells to grow and multiply abnormally. Medical researchers don’t know exactly what triggers these changes, but they have identified several factors that may increase risk.^[2][4]

For chronic lymphocytic leukaemia, risk factors include:^[2][4]

• Age: CLL typically affects people aged 65 and older, though it can occur starting at age 30
• Sex: CLL is more common in males than females
• Family history: People with close relatives who have CLL, such as a parent, sibling, or child, are two to four times more likely to develop the condition
• Exposure to Agent Orange: Studies show links between CLL and this chemical used during the Vietnam War
• Monoclonal B-cell lymphocytosis: Having a higher-than-normal number of identical B cells in the blood increases risk

CLL is more common in white people and particularly affects white men.^[4]

Diagnosis

Diagnosis of lymphocytic leukaemia often begins with a physical examination. The doctor checks for swollen lymph nodes in the neck, underarms, and groin, and feels for an enlarged spleen or liver.^[6][12]

Several tests are used to diagnose the condition:^[6][10][12]

Blood tests are the primary diagnostic tool. A complete blood count measures the number of cells in a blood sample. In chronic lymphocytic leukaemia, there is usually a high number of B cells, with lymphocyte counts typically greater than or equal to 5,000 per cubic millimeter. A peripheral blood smear shows what cells look like under a microscope. In CLL, the smear may show many small, round lymphocytes, sometimes called smudge cells.^[12][15]

Flow cytometry is a test that identifies proteins on the surface of cancer cells. In CLL, the cells have specific protein markers, typically CD5-positive and CD23-positive B cells, that help identify them and predict how aggressive the disease may be.^[6][15]

Genetic testing looks for changes in the cancer cell DNA. Lab tests called fluorescence in situ hybridization (FISH) can show which DNA changes are present in the leukaemia cells. These changes help doctors understand the disease and plan treatment.^[12][15]

Bone marrow biopsy and aspiration may be recommended if blood counts are low without a clear reason. This procedure involves collecting cells from the bone marrow using thin, hollow needles. The cells are sent to a laboratory for testing.^[4][12]

Imaging tests such as CT scans may be used if there is extensive swelling of lymph nodes or concerns about internal organs, though they are not always required.^[6][12]

Treatment

Treatment for chronic lymphocytic leukaemia varies depending on how advanced the disease is and whether symptoms are present. Currently, healthcare providers don’t have routine treatments to cure CLL, but they do have treatments that can put it into remission, meaning you don’t have symptoms or signs of the disease. These treatments are helping people with CLL live longer.^[2][17]

Watch and wait approach

Many people with CLL do not need treatment right away. Research shows that treating CLL before there are certain signs of disease progression doesn’t help people live longer. Because treatments can cause side effects, doctors often start with a “watch and wait” approach, also called active surveillance.^[1][13][17]

During active surveillance, your healthcare team uses tests and regular examinations to check if CLL is progressing or if your condition is getting worse. They also help and encourage you to take steps to stay healthy. You will be seen by a doctor or nurse regularly, though how often depends on the stage of your cancer and how you’re feeling.^[13][17]

When treatment begins

Treatment typically starts when symptoms develop or the disease shows signs of progression. When you start treatment depends on your symptoms, the stage of the cancer, and your general health.^[13][17]

Main treatment options include:^[1][13][17]

Targeted therapy uses drugs to target specific molecules on cancer cells. These medicines aim to stop the cancer from growing or help your immune system fight the cancer. You may have them on their own or combined with other treatments.

Chemotherapy uses medicines to kill cancer cells. It is usually given together with targeted cancer medicines.

Immunotherapy helps boost the body’s natural defenses to fight cancer.

Bone marrow transplant, also called stem cell transplant, may be used in certain situations.

Other options being studied in clinical trials include CAR-T cell therapy and newer immunotherapy approaches. Rarely, radiation therapy may be used if symptoms get worse or other treatments aren’t working well. Surgery to remove the spleen may be needed in some cases.^[1][17]

Supportive treatments

You may also need treatments to prevent or control symptoms caused by CLL. These may include:^[6]

• Medicines to prevent infections
• Vaccines to prevent illnesses such as flu, pneumonia, and COVID-19
• Blood or platelet transfusions
• Steroid medicines

Living with relapses

When symptoms come back after treatment, this is called a relapse. This may happen slowly and you may not need treatment straight away. You may have several relapses while living with CLL. When symptoms are under control, this is called remission, which can last for years.^[17]

When you need more treatment to control the cancer, you’ll have additional tests so doctors can determine which treatments will work best for you.^[17]

Living with the condition

Living with chronic lymphocytic leukaemia requires adjustments, but it doesn’t have to change everything about your life. Taking time for yourself to relax and manage stress is a valuable part of your recovery and overall well-being.^[18]

Emotional support

It’s normal to have many different feelings after being told you have cancer. You might feel shocked, upset, frightened, uncertain, confused, angry, guilty, or sad. You may have some or all of these feelings, or you might feel totally different. These emotions can leave you feeling exhausted.^[19]

Talking to friends and relatives about your cancer can help support you, though some people may find it difficult to discuss. You might also find it easier to talk to someone other than your own family and friends, such as a counselor or support group. Many organizations offer support services where you can connect with others affected by cancer.^[19]

Staying healthy

Focusing on certain lifestyle changes can help you cope better with treatment side effects and improve your long-term health. These include:^[19][20]

• Eating a varied and nutritious diet
• Being physically active with regular exercise
• Taking care of your mental health
• Getting adequate rest and sleep
• Staying up to date with recommended vaccinations

Practical matters

Getting a lot of new information can feel overwhelming. It helps to:^[19]

• Make lists to help you remember things and questions for your healthcare team
• Keep a calendar with all appointments
• Set achievable goals
• Plan enjoyable activities
• Ask for help when you need it

Remember that you don’t have to sort everything out at once. It may take time to deal with each issue. Your healthcare team is there to support you throughout your journey with this condition.^[19]