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Chronic Lymphocytic Leukaemia

CLL

Leukaemia is a cancer of cells in the bone marrow (the cells which develop into blood cells). With leukaemia, the cancerous cells made in the bone marrow spill out into the bloodstream. There are several types of leukaemia. Most types arise from cells which normally develop into white blood cells.

What is normal blood made up of?

  • Red cells (erythrocytes). These make blood a red colour. One drop of blood contains about five million red cells. Red cells contain a chemical called haemoglobin. This binds to oxygen, and takes oxygen from the lungs to all parts of the body.
  • White cells (leukocytes). There are different types of white cells which are called neutrophils (polymorphs), lymphocytes, eosinophils, monocytes and basophils. They are part of the immune system. Their main role is to defend the body against infection.
  • Platelets. These are tiny and help the blood to clot if we cut ourselves.
  • Plasma is the liquid part of blood and makes up about 60% of the blood's volume. Plasma is mainly made from water, but contains many different proteins and other chemicals, such as hormones, antibodies, enzymes, glucose, fat particles, salts, etc.

Bone marrow

Blood cells are made in the bone marrow by 'stem' cells. The bone marrow is the soft 'spongy' material in the centre of bones. The large flat bones, such as the pelvis and breastbone (sternum), contain the most bone marrow.

Stem cells

Stem cells are primitive (immature) cells. There are two main types in the bone marrow - myeloid and lymphoid stem cells. Stem cells constantly divide and produce new cells. Some new cells remain as stem cells and others go through a series of maturing stages ('precursor' or 'blast' cells) before forming into mature blood cells. Mature blood cells are released from the bone marrow into the bloodstream.

Lymphocyte white blood cells develop from lymphoid stem cells. There are three types of mature lymphocytes:
B lymphocytes make antibodies which attack infecting bacteria, viruses, etc.
T lymphocytes help the B lymphocytes to make antibodies.
Natural killer cells, which also help to protect against infection.

All the other different blood cells (red blood cells, platelets, neutrophils, basophils, eosinophils and monocytes) develop from myeloid stem cells.

The main types of leukaemia are:

  • Acute lymphoblastic leukaemia (ALL).
  • Chronic lymphocytic leukaemia (CLL).
  • Acute myeloid leukaemia (AML).
  • Chronic myeloid leukaemia (CML).

What is chronic lymphocytic leukaemia?

CLL is a condition where you have many abnormal B lymphocytes. The lymphocytes look normal under a microscope, but are abnormal as they do not function properly. The main reason for the build-up of the abnormal lymphocytes is because they live too long - they do not die after the usual lifespan of a lymphocyte.

What causes chronic lymphocytic leukaemia?

A leukaemia is thought to first start from one abnormal cell. What seems to happen is that certain vital genes which control how cells divide, multiply and die, are damaged or altered. This makes the cell abnormal.

What are the symptoms of chronic lymphocytic leukaemia?
At first, in many cases, there are no symptoms. In time, the abnormal lymphocytes may fill much of the bone marrow. Because of this, it is difficult for normal cells in the bone marrow to survive and make enough normal blood cells. Therefore, the main problems which may eventually develop include:

  • Anaemia.
  • Blood clotting problems.
  • Serious infections.

The abnormal lymphocytes may also build up in lymph glands and in the spleen. With CLL it is also common to develop swollen glands in various parts of the body, particularly in the neck and armpits, and develop an enlarged spleen. Some people with CLL also have an associated autoimmune disease.

Transformation of CLL

In about 1 in 6 cases, the CLL changes at some point (transforms) to a more 'acute' form of leukaemia. This then progresses more rapidly and responds less well to treatment.

How is chronic lymphocytic leukaemia diagnosed and assessed?

A blood test
Cell typing (immunophenotyping)
Detailed tests are done on the lymphocytes obtained from the blood test (or bone marrow sample). These help to confirm the diagnosis of CLL and to rule out other rarer related disorders.
A bone marrow sample
This test is not always necessary to diagnose CLL. It may be done to confirm the diagnosis and rule out other conditions.

Various other tests

A chest X-ray, blood tests and other tests may be done to assess your general well-being.
Staging chronic lymphocytic leukaemia

  • Stage A - there are fewer than three areas in the body with swollen lymph glands.
  • Stage B - there are three or more areas in the body with swollen lymph glands.
  • Stage C - there is a low number of red cells (anaemia), a low number of platelets, or both.

What is the treatment for chronic lymphocytic leukaemia?

Treatment for stage A (early stage)

Many people with stage A CLL do not need any treatment. This is because many people with stage A disease do not have any symptoms, and it often causes little harm. In many cases of stage A disease, the disease progresses slowly and never needs treatment.

Treatment for stage B and C (later stages)

The aim of treatment is to kill the abnormal cells. This then allows the bone marrow to function normally again, and produce normal blood cells. The main treatment is chemotherapy.

Chemotherapy is a treatment which uses anti-cancer medicines to kill cancer (leukaemia) cells, or to stop them from multiplying. For CLL, the chemotherapy is usually taken as tablets which you take each day. Sometimes chemotherapy medicines are given by injection.

Monoclonal antibodies are a fairly new treatment which is sometimes used to treat CLL - for example, products called alemtuzumab and rituximab. Monoclonal antibodies are small proteins and are different to normal chemotherapy. They work by attaching to the abnormal lymphocytes, which destroys them without harming other cells. Monoclonal antibodies may be used in addition to chemotherapy.

Radiotherapy may be used to reduce the size of enlarged lymph glands or spleen.

Steroid tablets are sometimes added to the treatment. They may help the chemotherapy medicines to work better.

A stem cell transplant

A stem cell transplant (SCT) - sometimes called bone marrow transplant - is sometimes used, especially in younger patients with progressing disease. It involves intense chemotherapy. A successful SCT can cure CLL, as the lymphocytes made by the transplanted stem cells will be normal.

What is the outlook (prognosis)?

Overall, the outlook is reasonably good. Most patients are able to enjoy a good quality of life for many years, with little or no treatment. Although CLL is not a condition that can usually be cured, many cases are in stage A where the condition is not usually serious, and treatment is not usually needed. Treatment for stages B and C often puts the disease into remission. Your specialist will be able to give a more accurate prognosis for your particular circumstances.


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