Chronic lymphocytic leukaemia (CLL)

Published by Bupa's Health Information Team, December 2010.

This factsheet is for people who have chronic lymphocytic leukaemia, or who would like information about it.

Chronic leukaemia is a cancer of the blood that develops slowly. Chronic lymphocytic leukaemia (CLL) affects a type of white blood cell known as lymphocytes. It is the most common type of leukaemia and tends to affect people over the age of 65.

About chronic lymphocytic leukaemia

The term leukaemia refers to a group of cancers of the blood cells and bone marrow. If you have leukaemia, your white blood cells become abnormal and divide and grow in an uncontrolled way.

Leukaemia can be described as either acute (growing rapidly) or chronic (growing slowly). Chronic leukaemia is divided into two main types, depending on the type of cells affected – CLL and chronic myeloid leukaemia (CML). This factsheet focuses on CLL.

CLL affects a type of white blood cell known as lymphocytes. Lymphocytes are produced by your bone marrow to fight off infection. In CLL, your body produces abnormal lymphocytes, which are unable to carry out their normal function. They also build up in your bone marrow, so your body may not be able to make enough of other types of blood cell (red cells, neutrophils and platelets).

About 2,300 people are diagnosed with CLL each year. It tends to affect people who are between 65 and 70 years of age and is rare in people under 40.

Symptoms of chronic lymphocytic leukaemia

Symptoms of CLL include:

• feeling very tired and breathless – this is due to anaemia (your body not making enough red blood cells)
• swollen lymph nodes (glands in your neck, groin and under your arms)
• fever and sweating attacks at night
• weight loss
• pain or discomfort in your abdomen (tummy) due to an enlarged spleen
• increased bruising or unusual bleeding (eg frequent nosebleeds, bleeding from gums and cuts) – this is due to not having enough platelets in your blood
• having frequent infections, such as pneumonia and shingles, which take longer to recover from

These symptoms aren't always due to CLL, but if you have them, see your GP. Many of the symptoms are vague and can be confused with common illnesses like flu. In fact, one in four people with CLL don’t have any symptoms at all but CLL is picked up during a routine blood test.

Causes of chronic lymphocytic leukaemia

The exact reasons why you may develop CLL aren’t fully understood at present. However, doctors do know that there are several risk factors for developing CLL.

Risk factors include:

• age – CLL is most common in people over 60
• being male – you’re twice as likely to develop CLL if you’re a man
• a family history of CLL
• a weakened immune system – for example if you have HIV/AIDS or you’re taking medicines that suppress your immune system

Diagnosis of chronic lymphocytic leukaemia

Your GP will ask about your symptoms and examine you. He or she may ask you to have a blood test to check the levels of different cells in your blood. If your GP thinks that you may have CLL, he or she will refer you to a specialist called a haematologist (a doctor who specialises in conditions of the blood).

The haematologist will perform some further investigations to confirm the diagnosis of CLL. These may include:

• a physical examination to check whether your spleen, lymph nodes or liver are enlarged
• a bone marrow biopsy – your doctor will remove a sample of your bone marrow, which will be examined under a microscope
• specific tests on the lymphocytes in your blood to determine their exact nature (immunophenotyping)
• genetic analysis of the chromosomes in the lymphocytes (cytogenetics)
• further blood tests, to check the level of antibodies (immunoglobulins) in your blood
• a CT scan of your whole body to look more accurately for enlargement of your lymph nodes and spleen

These tests are all very important because they help to determine how far your leukaemia has progressed and which treatment is best for you.

Staging for chronic lymphocytic leukaemia

You may hear your doctors referring to stages of cancer. These stages measure how far your leukaemia has progressed so that you’re provided with the most appropriate treatment. The Binet staging system is generally used in the UK and the rest of Europe.

Binet stage A
If you have stage A, you will have an increased number of lymphocytes in your blood, but a normal level of the other blood cells. There will be less than three areas of enlarged lymph nodes in your body. You will not usually need treatment if you have stage A CLL.

Binet stage B
This is when you have a high lymphocyte count and three or more areas of enlarged lymph nodes in your body. You may not have any symptoms or feel tired.

Binet stage C
At this stage, you will have a high lymphocyte count and either a low red blood cell count or low platelet count, or both. Your lymph nodes and spleen may be bigger than usual. You may be able to feel your swollen spleen to the left of your stomach under your ribs, and you are more likely to feel unwell.

Transformation
Sometimes CLL can transform or change itself into a lymphoma (cancer of your lymphatic system) called Richter’s syndrome. This happens in about one in 20 people with advanced CLL.

Treatment of chronic lymphocytic leukaemia

If you are diagnosed with stage A CLL, you are unlikely to need any treatment at first. However, your doctor will want to check your blood count regularly to monitor the progress of your condition. CLL develops very slowly, so many people with the disease will never require any treatment.

You may be offered one or more of the following treatments if your condition progresses, or if you start to have more symptoms. The aim of treatment is to reduce the number of CLL cells in your blood, so that you are free of symptoms. This is called remission. Remission can last for a number of years, and you can be given more treatment if your cancer starts to grow again.

Chemotherapy

Chemotherapy uses medicines to destroy cancer cells in your bone marrow. This is the main treatment used for stages B and C of CLL. You will be given a combination of medicines called fludarabine and cyclophosphamide or chlorambucil on its own. You may be given these as tablets to take by mouth, or through a drip in your vein.

Steroids

Your doctor may prescribe steroids to take on their own, or at the same time as chemotherapy. They can be effective at treating CLL as well as some of the complications of the condition. Examples of these medicines include prednisolone and dexamethasone. Steroids can cause some side-effects including heartburn and muscle weakness.

Radiotherapy

Radiotherapy uses X-rays to target cancer cells. You may have radiotherapy if you have an enlarged spleen or lymph nodes that are causing you problems.

Bone marrow (stem cell) transplant

This is when you have a transplant of healthy bone marrow or stem cells from somebody else (preferably a brother or sister) into your body. Your doctor may offer you a bone marrow (stem cell) transplant if you are young and in good health. However, there’s no evidence yet about how helpful this is at treating CLL.

Biological therapies

Biological therapies are medicines that are designed to mimic or inhibit our natural body substances, such as antibodies, to help fight cancer cells. Rituximab and alemtuzumab are both a type of biological therapy known as monoclonal antibodies, which are used in the treatment of CLL. They work by seeking out and destroying CLL cells in your bone marrow. You may be given these medicines alongside chemotherapy or after your chemotherapy is over.

Immunoglobulin

In addition to other treatments, your doctor may also suggest regular monthly infusions of a type of antibody, called human normal immunoglobulin by drip to help build up your immune system.

New treatments

New potential treatments being investigated for leukaemia include new chemotherapy medicines, mini-stem cell transplants, cord-blood cell transplants and biological therapies. They are all being tested in clinical trials. Many people with leukaemia take part in clinical trials as new treatments are constantly changing. Your doctor can give you more information about clinical trials.

For answers to frequently asked questions on this topic, see Common questions.

For sources and links to further information, see Resources.