Chronic lymphocytic leukaemia

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.

The blood

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.

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.

There are two types of chronic leukaemia depending on what type of white blood cell is involved:

• chronic lymphocytic leukaemia (CLL)
• chronic myeloid leukaemia (CML).

CLL affects the lymphocytes, which are a type of white blood cell. CLL is also sometimes known as chronic lymphatic leukaemia.

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.

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:

• tiredness
• sweating
• 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.

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.

A bone marrow biopsy is an uncomfortable procedure and can be painful. You will be given a local anaesthetic to numb the area. The procedure usually lasts up to 30 minutes. There are a wide range of pain relief medications available. Ask you doctor what is suitable for you before the biopsy. You may be asked to fast (not eat or drink) prior to the procedure. If you have sedation for the procedure this will make you drowsy so you will need someone to drive you home.

Lymph gland biopsy

Occasionally, to confirm the diagnosis, one enlarged lymph gland is removed. This is called a lymph gland 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.

CT scan

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.

A CT scan takes about 30 minutes.

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.

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.

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 or a low platelet count or both.

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 a curable disease but, for many people, treatment can control it for years and allow them to live a normal life.

Your GP will arrange the initial blood test to check out your symptoms. If CLL is suspected, you will be referred to other health professionals, including:

• Haematologist: diagnoses and treats diseases of the bone marrow, blood and lymphatic system
• Radiation oncologist: prescribes and co-ordinates the course of radiotherapy
• Nurses: support and assist you through all stages of your treatment
• Other health professionals that you may also see include:
• Dietician: recommends the best eating plan to follow while you are in treatment and recovery
• Social worker, physiotherapist and occupational therapist: link you to support services and help you to resume your normal activities.

Chemotherapy uses drugs, called cytotoxic, to kill or slow the growth of leukaemia cells.

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. The commonly used chemotherapy drugs are chlorambucil and cyclophosphamide. Some people may receive chemotherapy by injection into a vein (intravenously).

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.

Purine analogues are a group of drugs known as anti-metabolites. They 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.

These drugs are usually given intravenously or under the skin.

Fludarabine is commonly used in treatment of CLL and follicular lymphoma.

Biological therapy, which includes immunotherapy, uses materials made by the body’s immune system or materials made in a laboratory to stimulate the body to fight infection.

Immunotherapy for CLL includes a protein called gamma interferon, which is made by the white blood cells.

Another type of immunotherapy is antibodies. Antibodies work by finding and becoming attached to targeted cells such as lymphocytes and then destroying them.

Monoclonal antibodies, which are made in the laboratory, can be given intravenously.

The monoclonal antibody available in Australia and used to treat CLL is alemtuzumab (CamPath).

Another biological therapy is called granulocyte-colony stimulating factor (G-CSF), which helps the white cells to recover faster after chemotherapy. It is given under the skin after chemotherapy. You may have the treatment on its own intravenously, or with chemotherapy.

Bone marrow transplantation involves very high doses of chemotherapy.

The chemotherapy will destroy the bone marrow but transplanted stem cells restore blood cell numbers to normal.

Stem cells are early-stage cells from which other cells develop.

Sources of stem cells

• Bone marrow
• Blood – these are called peripheral stem cells

Donors of stem cells

• from another person: allogeneic transplant.
• your own: autologous transplant.

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.

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.