This information has been written to help you understand more about chronic lymphocytic leukaemia.
Many people feel understandably shocked and upset when they are told they have chronic lymphocytic leukaemia.
This information is intended to help you understand the diagnosis and treatment of the disease. We also include information about support services.
We cannot advise you about the best treatment for you. You need to discuss this with your doctors.
The bone marrow
The bone marrow is the soft spongy material in the centre of bones. The bone marrow makes early stage cells called stem cells, from which all other blood cells develop.
There are two types of stem cells:
- Myeloid stem cells develop into three types of blood cells: red, white and platelets.
- Lymphoid stem cells develop into other types of white blood cells called T-lymphocytes and B-lymphocytes.
When stem cells mature, they are released into the bloodstream.
Blood is pumped around your body to provide oxygen, nutrients, fight infections and remove waste products. The three main types of blood cells are carried along in a clear fluid called plasma.
- Red blood cells: Carry oxygen from your lungs to the rest of your body. If you don’t have enough red cells, you may be pale and tire easily because your body is not getting enough oxygen. A shortage of red blood cells is called anaemia.
- White blood cells: Fight infections. If you do not have enough white cells, your risk of infection increases.
- Platelets: Help your blood clot and stop bleeding. If you do not have enough platelets, you may bruise for no known reason, have frequent nose bleeds or prolonged bleeding, or develop a rash of small dots to the arms, legs or body.
What is chronic leukaemia?
Chronic leukaemia develops when your white blood cells grow out of control. The abnormal white cells live too long, so there are too many circulating in the blood. These white cells are not fully developed and do not work properly.
The bone marrow becomes crowded with abnormal white cells, leaving little room for healthy red cells and platelets to be produced.
Chronic leukaemia appears gradually and develops slowly over months to years.
Types of chronic leukaemia
There are two types of chronic leukaemia depending on what type of white blood cell is involved:
Chronic myeloid leukaemia (CML): affects a type of white blood cell called granulocytes. Granulocytes are part of the myeloid family of white blood cells.
Chronic lymphocytic leukaemia (CLL): affects white blood cells called lymphocytes. CLL is also sometimes known as chronic lymphatic leukaemia.
How common is it?
In 2003 in NSW, 44% of the 883 new cases of leukaemia were chronic leukaemia.
CLL is more common among people over 60 years of age and is rare in people under 40.
What is the cause?
The cause of CLL is not known but research is trying to find out why it develops. Some people may have genetic or chromosomal abnormalities that can cause CLL.
What are the symptoms?
Many people with chronic lymphocytic leukaemia have no symptoms. The illness is difficult to detect in the early stages because the symptoms tend to be mild at first and get worse slowly.
Symptoms of chronic lymphocytic leukaemia include:
- weight loss
- lump in the left side of the stomach from an enlarged spleen
- swollen lymph glands
- bruising or bleeding from a low platelet count.
Occasionally, a person will have none of these symptoms and the leukaemia is discovered during a routine blood test.
Tests to diagnose chronic lymphocytic leukaemia
Your doctor will often do a number of tests before they diagnose chronic lymphocytic leukaemia.
An initial blood test will show if leukaemia cells are present or if the levels of blood cells are different to those of a healthy person. Other tests will confirm a diagnosis and will determine what type of leukaemia you have.
This test is usually done to confirm if leukaemia cells are present and to work out which leukaemia you have.
The bone marrow sample is examined for cell and chromosome changes, which in leukaemia cells are different to the normal cells. These changes also influence the type of treatment your doctor recommends for you.
A thin needle is used to remove a small piece of bone and bone marrow fluid from the hipbone.
Occasionally, to confirm the diagnosis, one enlarged lymph gland is removed. This is called a lymph gland or lymph node biopsy.
The gland is removed with a local or general anaesthetic and sent to the laboratory for testing. Most people go home the same day. After the biopsy, you will have a few stitches.
Some people with CLL may need a CT (computerised tomography) scan. This test uses x-ray beams to take pictures of the inside of your body. It can see if the lymph nodes are affected and if your spleen is enlarged.
Before the scan, a special dye may be injected into a vein, probably in your arm. This will make the pictures the scan takes clearer. For a few minutes, this may make you feel hot all over.
A CT scan takes about 30 minutes. You will lie flat on a table while the CT scanner, which is large and round like a doughnut, takes pictures.
Most people are able to go home as soon as their scan is done.
Staging chronic lymphocytic leukaemia
The results of these tests will be used to see how far the chronic lymphocytic leukaemia has spread. This is called staging.
Your treatment will depend on the stage of your leukaemia. There are three stages.
Early: High white blood cell count. No enlarged lymph nodes.
Intermediate: High white blood cell count and enlarged lymph nodes.
Late: Enlarged lymph glands and low red blood cell count, a low platelet count and/or an enlarged liver or spleen.
CLL usually develops slowly, However, in a small number of people, CLL can suddenly begin to develop more quickly. This is called transformation.
The first sign of transformation may be a worsening of your symptoms: high temperature, weight loss or sudden swelling of affected lymph nodes, especially in your abdomen.
Prognosis means the expected outcome of a disease.
You need to discuss the prognosis of your chronic leukaemia with your doctor. Only someone who knows your medical condition can tell you what to expect and the treatment options that are best for you.
CLL is not generally a curable disease but, for many people, treatment can control it for years and allow them to live a normal life.
Which health professionals will I see?
You will be cared for by a range of health professionals who specialise in different aspects of your treatment. Health professionals who may care for you include:
- General practitioner (GP) – arranges the first tests to investigate your symptoms.
- Haematologist – a doctor who treats people with blood diseases.
- Medical oncologist – prescribes and coordinates targeted therapies and chemotherapy.
- Radiation oncologist – prescribed and coordinates radiotherapy.
- Nurses – support you through all stages of your cancer treatment.
- Cancer care coordinator or clinical nurse consultant (CNC) – supports patients and families throughout treatment and liaises with other staff.
- Dietician – recommends an eating plan to follow while you’re in treatment and recovery.
- Social workers, counsellors, physiotherapists and occupational therapists – link you to support service social worker, and help with emotional, physical or practical issues.
Treatment usually begins as soon as you have been diagnosed and will depend on what type of chronic leukaemia you have. You will probably have chemotherapy and you may have radiotherapy. You may also need steroid therapy, or a peripheral blood stem cell or bone marrow transplant, depending on the type of leukaemia you have and your response to chemotherapy. In cases of advanced cancer, you may have palliative care.
Some chronic leukaemias grow slowly and cause few problems. Your doctor may recommend no treatment but keep an eye on the progress of the leukaemia with regular checkups and blood tests.
People with CLL need no treatment if they have no enlarged lymph glands or rising number of white blood cells.
See your doctor immediately if you develop viral or other infections such as shingles, which can occur in CLL. You’ll be taught how to recognise the signs so you can get early treatment. Your doctor may recommend you have a yearly flu vaccination.
If the CLL is diagnosed at an intermediate or late stage, you may have chemotherapy. This treatment uses anti-cancer drugs called cytotoxics to kill cancer cells or slow their growth.
Chemotherapy mainly kills fast-growing cells such as leukaemia cells. Other fast-growing cells, such as the cells involved in hair growth and cells in the mouth, can also be affected. This can make your hair fall out or cause mouth ulcers.
Most people with CLL are treated with chemotherapy tablets, which are usually taken for about seven days each month. Some people may receive chemotherapy by injection into a vein (intravenously). You may also have a combination of 2-3 different chemotherapy drugs.
The major types of chemo drugs used to treat CLL include:
Purine analogues include fludarabine and cladribine. These drugs stop cells making and repairing DNA and RNA (the cell’s genetic code). Cells need DNA and RNA to live and multiply. Fludarabine is often one of the first drugs used against CLL.
Alkylating agents , which include chlorambucil (Leukeran®) and cyclophosphamide (Cytoxan®), are often used along with a purine analog, with other chemo drugs, with a corticosteroid, or with the monoclonal antibody therapy.
Some drugs are designed to attack specific types of cells. A common type of targeted treatment for CLL is monoclonal antibodies, which can be given along with chemotherapy drugs. Monoclonal antibodies bind to certain cells, including leukaemia cells, and either cause those cells to die or make them more susceptible to being killed by the body’s own immune system.
The most common side effects of monoclonal antibodies are an increased risk of infection,fever, sweating, chills and diarrhoea. Your doctor will talk to you about the side effects, and you may be prescribed medication to ease them.
The monoclonal antibody available in Australia and used to treat CLL is alemtuzumab (CamPath).
Bone marrow or stem cell transplantation
Bone marrow transplantation involves very high doses of chemotherapy. A reduced intensity stem cell transplant (sometimes called a ‘mini’ transplant) may be performed for patients who haven’t responded to chemotherapy. This means lower doses of chemotherapy and radiotherapy are used for the transplant, so it is easier for the body to tolerate.
Steroids are made naturally in the body. They can also be made artificially and used as drugs. In CLL, steroid therapy is sometimes given with chemotherapy.
Radiotherapy uses radiation, usually x-rays or gamma rays, to kill cancer cells or injure them so they cannot multiply. The x-rays are aimed at areas of chronic leukaemia cells using an external machine. This treatment may be considered if lymph glands have not shrunk after chemotherapy treatment.
Treatment is carefully planned to destroy cancer cells with as little harm as possible to your normal tissues. You will lie under a machine, which takes x-ray pictures. Each treatment only takes a few minutes once started, but it can take between one and three hours to set up the machine, see the radiation oncologist and have blood tests.
You will probably have treatment from Monday to Friday for several weeks. The length of treatment will depend on the size and type of cancer and on your general health.
Removing the spleen
The spleen is part of the immune system and helps fight infection. CLL may cause the spleen to become enlarged, causing discomfort and pain.
An operation to remove the spleen is called a splenectomy.
A splenectomy takes a couple of hours and requires a hospital stay of 4-7 days. Side effects include increased risk of infections.
Palliative treatment helps improve quality of life by alleviating the symptoms of cancer without trying to cure the disease. It is particularly important for people with advanced cancer.
Often treatment is concerned with symptom control and stopping the spread of cancer, but it can also involve the management of other physical and emotional symptoms. Treatment may include chemotherapy or medication.
After your treatment is over, you will need regular checkups with your doctor or your cancer treatment centre. Blood tests will be done to check your general health and your blood count. Regular checkups can help find a recurrence early, and this gives you the best chance of getting the disease under control.
Checkups will continue for several years but will become less frequent if you have no further problems.
Between follow-up appointments, let your doctor know immediately of any health problems.
What if the leukaemia returns?
Some people find their leukaemia comes back. This is called relapse.
If you have a relapse, further treatment can be given — usually using a different combination of chemotherapy drugs to those you had before. Often this can lead to a second remission. If you have not had a transplant before, your doctors may recommend a bone marrow or stem cell transplantation.
Understanding chronic lymphocytic leukaemia
A guide for people with cancer, their families and friends.