Page last reviewed: 13/07/2011
Leukaemia is cancer of the white blood cells. Symptoms of leukaemia include:
- pale skin
- repeated infections over a short space of time
Chronic leukaemia means that the condition progresses slowly over the course of many years and no immediate treatment is required.
These pages focus on chronic lymphocytic leukaemia
All of the blood cells in the body are produced by bone marrow. Bone marrow is a spongy material found inside the bones. It is important because it produces special cells called stem cells.
Stem cells are very useful because they have the ability to create other specialised cells that carry out important functions. The stem cells in bone marrow produce three important types of blood cells:
- red blood cells, which carry oxygen around the body
- white blood cells, which help fight infection
- platelets, which help stop bleeding
In leukaemia, the cancer begins in the stem cells and causes them to produce more white blood cells than are needed. Over time, the cancerous white blood cells build up, disrupting the normal balance of cells in the blood.
This means that the body does not have enough red blood cells or platelets. This can cause symptoms of anaemia, such as tiredness, as well as increasing the likelihood of excessive bleeding.
Also, as the white blood cells are not properly formed, they are less effective at fighting bacteria and viruses, making you more vulnerable to infection.
Types of chronic leukaemia
Chronic leukaemia is classified according to the type of white blood cells that are affected by cancer. There are two main types of white blood cell:
- lymphocytes - which are mostly used to fight viral infections
- myeloid cells - which perform a number of different functions, such as fighting bacterial infections, defending the body against parasites and preventing the spread of tissue damage
There are two main types of chronic leukaemia that are related to the two main types of white blood cells. They are:
- chronic lymphocytic leukaemia - which is cancer of the lymphocytes
- chronic myeloid leukaemia - which is cancer of the myeloid cells
These pages focus on chronic lymphocytic leukaemia (CLL). For information on chronic myeloid leukaemia,
How common is chronic lymphocytic leukaemia?
Chronic leukaemia is an uncommon type of cancer.
CLL is more common in older people, with most cases developing in people over the age of 55.
Chronic leukaemia can be cured using a bone marrow transplant. However, this is not suitable for everyone.
The outlook for CLL has improved dramatically in the last number of years depending on the stage of the cancer at the time of diagnosis and how well the patient respons to treatment The majority of patients do not need treatment immediately
Page last reviewed: 13/07/2011
In its early stages, chronic lymphocytic leukaemia (CLL) usually causes no noticeable symptoms.
As the condition develops, symptoms include:
- repeated infections (that occur over a short space of time)
- night sweats
- unusual bleeding and bruising
- swollen spleen
- swollen lymph nodes
The presence of swollen lymph nodes provides a useful way of tracking the progression of CLL. This is because as the number of lymphocytes increases, they begin to collect in your lymph nodes, which are part of your lymphatic system.
The lymphatic system is a series of glands (or nodes) that are spread throughout your body in a similar way to your blood circulation system. These glands produce many of the specialised cells that are needed by your immune system.
Having three or more swollen lymph nodes may mean that your CLL has progressed and you may need treatment.
Page last reviewed: 13/07/2011
Chronic leukaemia begins with an alteration to the structure of the DNA found in the stem cells that are responsible for producing white blood cells. The DNA provides the cells with a basic set of instructions, such as when to grow and reproduce. The alteration that occurs to the DNA's structure is known as a genetic mutation.
The mutation in the DNA changes the instructions, which causes the stem cells to produce more white blood cells than are needed. The white blood cells are also produced too quickly and, over time, they begin to lose their infection-fighting properties.
Eventually, the increase in the number of affected white blood cells will lead to a corresponding decrease in red blood and platelets. The reduction in the other blood cells is an underlying cause for many of the symptoms of chronic leukaemia.
Possible triggers for chronic leukaemia
What triggers the development of chronic leukaemia and causes the initial mutation in stem cells is unknown. The one proven risk factor is exposure to radiation.
However, radiation is only a significant risk if the levels are extremely high, such as those recorded after an atomic bomb explodes, or those released after a nuclear reactor accident, such as the one at Chernobyl.
Chronic lymphocytic leukaemia (CLL) seems to have a genetic basis.
For example, a number of families have had higher-than-expected incidences of CLL, which suggests that a susceptibility to the condition runs in families.
And research has found that you are six to nine times more likely to develop CLL if you have a first-degree relative (mother, father, brother or sister) with CLL. However, exactly which genes are linked to CLL is still uncertain.
There are a number of occupations that have been linked to an increase risk of chronic leukaemia, possibly due to exposure to certain substances such as pesticides or chemicals.
These occupations include:
- all types of agricultural workers
- people involved with rubber or plastic manufacture
- tailors and dressmakers
- builder's labourers
Page last reviewed: 13/07/2011
Suspected cases of chronic leukaemia are usually first detected after a blood test, which has often been carried out to diagnose another, unrelated condition.
A blood test that reveals abnormally high levels of white blood cells could be a sign of chronic leukaemia. If you have a blood test with abnormal results, you will be referred to a haematologist (a specialist in treating blood conditions) for further testing.
Bone marrow biopsy
To confirm a diagnosis of chronic leukaemia, the haematologist will take a small sample of your bone marrow to examine under a microscope. This procedure is known as a bone marrow biopsy. A bone marrow biopsy is usually carried out under a local anaesthetic.
The haematologist will numb an area of skin at the back of your hip bone, before using a needle to remove the bone marrow sample. The procedure is usually painless although you may experience some bruising and discomfort for a few days afterwards. The procedure takes around 15 minutes to complete and you should not have to stay in hospital overnight.
The bone marrow sample will be checked to see if there are cancerous cells. If there are, the biopsy will also be able to help determine which type of chronic leukaemia is present.
There are a number of additional tests that can be used to help reveal more information about the progress and extent of the leukaemia, which can also provide an important insight into how the leukaemia should be treated. These are outlined below.
Cytogenetic testing involves identifying the genetic make-up of the cancerous cells. There are a number of specific genetic variations that can occur during leukaemia and knowing what these variations are can have an important impact on treatment.
Lymph node biopsy
If you have been diagnosed with chronic leukaemia, further biopsies may be carried out on any enlarged lymph nodes that you have. These are to see how far the leukaemia has spread.
If you have chronic leukaemia, a computerised tomography (CT) scan may be used to check that your other organs, such as your heart and lungs, are healthy.
Page last reviewed: 13/07/2011
Your treatment plan
As chronic leukaemia is a complex condition, it is usually treated by a team of different healthcare specialists working together. This type of team is known as a multidisciplinary team (MDTs).
Members of your MDT may include:
- a haemato-oncologist (a specialist in the non-surgical treatment of leukaemia using techniques such as chemotherapy)
- a haemato-pathologist (a specialist in the study of cancerous blood cells)
- a pharmacist
- a social worker
- a psychologist
- a specialist cancer nurse (who will serve as the first point of contact between you and the other members of the MDT)
- a counsellor
There are several factors that will need to be taken into account when deciding on your treatment. They include:
- your age
- the extent of your cancer
- the likely progression of your cancer
- the possible side effects of treatment
No one will rush you into making a decision about your treatment, and you are free to talk to as many people as you want to, including your partner, family and friends.
Many people find that it can be helpful to write a list of questions at home to ask the MDT. Your MDT will be able to recommend what they feel are the best treatment options for you, but ultimately the decision will be yours.
The treatment of chronic leukaemia when the condition is in its early stages will depend on what type of chronic leukaemia you have. However, the treatments for both chronic myeloid leukaemia and chronic lymphocytic leukaemia (CLL) in their advanced stage are more or less the same.
Treating early-stage chronic lymphocytic leukaemia
There is no current effective medical treatment for early-stage chronic lymphocytic leukaemia (CLL).
Because of their associated side effects, possible treatments for CLL are only recommended once you begin to experience symptoms, or the leukaemia progresses to a potentially serious stage.
Instead, a policy of "watchful waiting" is recommended. This means that you will have regular blood tests so that the progress of your condition can be carefully monitored.
Treating advanced chronic lymphocytic leukaemia
Once chronic lymphocytic leukaemia has progressed to a more advanced stage, chemotherapy is the next treatment.
Chemotherapy tablets are usually used first because they have fewer and milder side effects than chemotherapy injections. Side effects include:
- skin rash
- increased vulnerability to infection
Chemotherapy can weaken your immune system, which helps protect you against infection. This is known as being immunocompromised.
If your symptoms persist or get worse, chemotherapy injections (intravenous chemotherapy) will need to be used. Intravenous chemotherapy causes more side effects than chemotherapy tablets and they tend to be more severe.
Side effects include:
- hair loss
These side effects should resolve after your treatment has finished, although there is a risk that infertility could be permanent.
Rituximab is a new type of medication that is increasingly being used in combination with chemotherapy to treat CLL. Rituximab is what is known as a monoclonal antibody.
Monoclonal antibodies are antibodies that are genetically engineered in a laboratory. They are designed to directly target and attack cancer cells. This is why monoclonal antibody therapy can be referred to as targeted therapy.
Rituximab works by targeting a protein that is found on the surface of cancer cells, and encouraging your immune system to attack the protein which, in turn, should kill the cancer cells.
Rituximab is administered directly into your vein over the course of several hours. This is known as an infusion.
Common side effects of rituximab include:
- flu-like symptoms, such as chills and a high temperature, while the medication is being given
In rare cases, rituximab can cause severe allergic reactions in some people. This is known as an infusion reaction and it can be fatal if left untreated.
Most infusion reactions occur within 24 hours of the first time that someone begins treatment. Therefore, it is likely that you will be closely monitored once your treatment begins. If you start to experience the symptoms of an infusion reaction, such as shortness of breath or chest pain, anti-allergy medicines such as corticosteroids can be used to help relieve your symptoms.
Bone marrow and stem cell transplants
The only available cure for chronic lymphoblastic leukaemia is to have a bone marrow or stem cell transplant.
Before transplantation can take place, the person receiving the transplant has to have aggressive, high-dose chemotherapy and radiotherapy to destroy any cancerous cells in their body.
This can put enormous strain on the body and can cause significant side effects and potential complications. Transplantations have better outcomes if the donor has the same tissue type as the person who is receiving the donation. The best candidate to provide a donation is usually a brother or sister with the same tissue type.
Due to these issues, transplantations are usually only successful when they are carried out in children and young people, or older people in good health, and there is a suitable brother or sister who can provide a donation.
In most cases of chronic lymphocytic leukaemia, the potential risks of transplantation far outweigh any benefit. For example, the chances of an elderly person with advanced chronic leukaemia surviving a bone marrow transplant can be as low as one in five.
However, you may have specific individual circumstances that mean that the benefits of treatment do outweigh the risks.
Deciding against treatment
As many of the treatments that are described in this section have unpleasant side effects that can affect your quality of life, you may decide against having a particular type of treatment.
This is entirely your decision and your MDT will respect any decision that you make. Pain relief and nursing care will be made available as and when you need it.
Page last reviewed: 13/07/2011
Being immunocompromised (having a weakened immune system) is a common complication of chronic leukaemia. There are two reasons for this:
- The lack of healthy white blood cells means that your immune system is less able to fight infection.
- Many of the medicines used to treat chronic leukaemia can weaken the immune system.
This means that you are more vulnerable to developing an infection, and that any infection you have has an increased potential to cause serious complications.
You may be advised to take regular doses of antibiotics to prevent infections occurring. You should immediately report any possible symptoms of an infection to your GP or care team because prompt treatment may be required to prevent serious complications.
Symptoms of infection include:
- high temperature (fever) of 38C (101.4F) or above
- aching muscles
Avoid contact with anyone who is known to have an infection, even if it is a type of infection that you were previously immune to, such as chickenpox or measles. This is because your previous immunity to these conditions will probably be suppressed (lowered).
While it is important to go outside on a regular basis, both for exercise and for your psychological wellbeing, avoid visiting crowded places and using public transport during rush hour.
Also ensure that all of your vaccinations are up-to-date. Your GP or care team will be able to advise you about this. You will be unable to have any vaccine that contains activated particles of viruses of bacteria such as:
- the mumps, measles and rubella (MMR) vaccine
- the polio vaccine
- the oral typhoid vaccine
- the BCG vaccine (used to vaccinate against tuberculosis)
- the yellow fever vaccine
Many treatments used to treat chronic leukaemia can cause infertility. In some cases, infertility may be permanent.
Your multidisciplinary team will be able to provide a good estimation of the risk of infertility in your specific circumstance.
It may be possible to take steps to guard against any risk of infertility before beginning treatment. Men can have samples of their sperm stored so that it can be used to implant an embryo. Similarly, women can have fertilised embryos stored, which can then be placed back into the womb after treatment.
An estimated 1 in 20 people with chronic lymphocytic leukaemia (CLL) will develop a secondary cancer of the lymphatic system. Exactly why this happens is still unclear.
The lymphatic system is made up of a series of vessels and glands, known as lymph nodes. These are spread throughout your body, much like your blood vessels.
The general medical term for cancers of the lymphatic system is lymphomas. The type of lymphoma that develops in people with CLL is known as Richter syndrome.
Symptoms of Richter syndrome include:
- sudden swelling of your lymph nodes
- a high temperature that is not caused by infection
- night sweats
- weight loss
- abdominal pain
Richter syndrome is treated with chemotherapy, rituximab and, less commonly, radiotherapy.
Psychological effects of chronic leukaemia
Receiving a diagnosis of chronic leukaemia can be very distressing, particularly if it is unlikely that your condition can be cured. At first, the news may be difficult to take in.
The situation can be made worse if you are confronted with the knowledge that even though your leukaemia may not currently be causing any symptoms, it could be a serious problem in later life. Having to wait many years to see how the leukaemia develops can be immensely stressful and can trigger feelings of anxiety and depression.
If you have been diagnosed with leukaemia, talking to a counsellor or psychiatrist (a doctor who specialises in treating mental health conditions) may help you to combat feelings of depression and anxiety. Antidepressants or medicines that help to reduce feelings of anxiety may also help you cope better with the condition.
You may find it useful to talk to other people who are living with leukaemia. Your GP or MDT may be able to provide you with details of local support groups.