Overview
Overall, leukaemia is the eighth most common form of cancer in Europe with about 81,000 new cases diagnosed each year.
Leukaemia is a form of cancer that develops from white blood cells. There are a number of different forms of leukaemia each with their own unique biological characteristics and treatment. Generally, the acute forms of leukaemia develop more quickly than the chronic or slow growing types (although acute crises can occur in chronic myeloid leukaemia).
Effective treatments are available for all forms of leukaemia and produce good results for the majority of patients.
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Key Figures
| Total number of new cancer cases in Europe (2006) |
3.2 million |
| Total number of cancer deaths in Europe (2006) |
1.7 million |
| Number of new leukaemia cases (2006) |
81,300 |
|
(44,800 men) |
|
(36,500 women) |
|
61,400 (EEA) |
|
(34,800 men) |
|
(26,600 women) |
|
59,700 (EU25) |
|
(33,800 men) |
|
(25,900 women) |
| Number of leukaemia deaths (2006) |
54,400 |
|
(29,300 men) |
|
(25,100 women) |
|
40,100 (EEA) |
|
(21,900 men) |
|
(18,200 women) |
|
39,400 (EU25) |
|
(21,500 men) |
|
(17,900 women) |
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Cancer Types
There are four basic types of leukaemia:
Acute Lymphoblastic Leukaemia (ALL)
ALL is rare but it is the most common form of cancer in children and can also affect teenagers and adults.
Acute Myeloid Leukaemia (AML)
AML affects the blood’s granulocytes which fill up the bone marrow preventing production of normal cells. This causes anaemia (lack of red blood cells) and bruising and bleeding (lack of blood platelets).
Chronic Lymphatic Leukaemia (CLL)
CLL is the most common form of leukaemia affecting the lymphocytes and tends to affect people over 50 years old.
Chronic Myeloid Leukaemia (CML)
CML affects white granulocytes which fail to mature or work properly and gradually fill up the bone marrow reducing the numbers of normal cells and platelets produced.
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Causes of Leukaemia
Acute Lymphoblastic Leukaemia (ALL)
ALL is not usually inherited, however some syndromes like Down’s syndrome and Fanconi’s syndrome are associated with an increased risk of developing leukaemia.
Acute Myeloid Leukaemia (AML)
Previous chemotherapy or radiotherapy treatment are known risk factors for developing acute myeloid leukaemia. In addition, benzene and other industrial solvents are also associated with a higher incidence of acute myeloid leukaemia.
Chronic Lymphatic Leukaemia (CLL)
CLL generally affects people over the age of 60 and is rare under the age of 40. It is not known what causes the damage which leads to abnormal lymphocytes developing into CLL.
Chronic Myeloid leukaemia (CML)
Exposure to high doses of radiation is a significant risk factor for developing CML. In addition, many patients with CML will have the Philadelphia chromosome. This is not an inherited gene but an abnormal chromosome which produces an enzyme (tyrosine kinase) which leads to the development of CML.
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Prevention of Leukaemia
There are no practical preventive measures which individuals can take to avoid being at risk of leukaemia.
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Common Symptoms
There are often no symptoms in the early stages of leukaemia. However, symptoms if they do occur may include:
- generalised weakness and fatigue - which might be due to anaemia
- infections - due to the reduced numbers of normal white cells in the blood
- weight loss
- bruising and bleeding - due to the reduced numbers of platelets in the blood
- bone and joint pain - usually due to bleeding into the joints
- breathlessness - due to anaemia and the reduced oxygen-carrying capacity of the blood
- enlarged lymph nodes
- an enlarged spleen – the spleen cannot normally be felt as it is under the ribs on the left side of the abdomen. However, if it is enlarged its lower edge will be below the ribs and can be felt
- discomfort in the abdomen - which may be due to the spleen being enlarged
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Diagnostic Tests
Diagnostic tests for leukaemias include:
- Blood film. A blood test will usually show a high number of white blood cells in the blood sample. In some types of leukaemia these will look abnormal, indicating the likelihood of leukaemia.
- Bone marrow biopsy. A bone marrow biopsy is normally taken to confirm a diagnosis of acute leukaemia. The sample is usually taken from the iliac crest and can be painful despite the use of a local anaesthetic.
- Cytogenetic testing. This identifies the genetic makeup of the leukaemia cells which can have important implications for treatment.
- Lymph node biopsy. If enlarged lymph nodes are found on clinical examination further biopsies are taken to check for development of the disease into the lymph nodes.
- CT Scans. A CT scan is used to check other organs including the heart and lungs.
- Lumbar puncture. If there is a risk that acute leukaemia has spread to the central nervous system, a lumbar puncture may be undertaken. Cerebrospinal fluid is examined for the presence of abnormal white cells.
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Treatment
Different treatments will be recommended according to:
- the type of leukaemia
- its stage
Acute Lymphoblastic Leukaemia (ALL)
Children
Treatment for children with acute lymphoblastic leukaemia usually comprises three phases: induction, consolidation and maintenance.
Initially, there will be about 100 billion leukaemia cells in the body. The induction treatment aims to destroy 99.9% of these cells to achieve a remission. This means that leukaemia cells will no longer be found in bone marrow, which will be able to return to normal. Routine blood tests will now also be normal. This is called remission. 95% of children with ALL will be in remission after one month of treatment.
The consolidation phase (lasting 4 to 8 months) and the maintenance phase (lasting up to two years) are designed to destroy any remaining but undetectable leukaemic cells. The exact treatment protocol will depend on a number of factors.
Intrathecal chemotherapy is a standard part of chemotherapy and may be given several times during the first 6 months of treatment. It is needed to ensure adequate levels of the drug reach the central nervous system as drugs given intravenously may not cross the blood-brain barrier
Some patients also receive radiotherapy to the brain and spinal cord to kill cells in the central nervous system. However, as this causes some problems with growth and development of the brain it is avoided where possible (especially in very young children).
Some children, such as those with Philadelphia chromosome positive ALL, may benefit from a stem cell transplant at this stage.
If the leukaemia returns, further phases of chemotherapy will be planned (probably including further intrathecal therapy).
For children who relapse within 6 months, a bone marrow transplant may be considered - especially if there is a sibling who is a good tissue type match.
Extramedullary relapse means that leukaemic cells are found in one part of the body, usually the CSF or a boy’s testicles, but are not found in the bone marrow. This relapse is usually treated with radiotherapy to the affected area.
Adults
As for children with acute lymphoblastic leukemia, treatment of adults usually comprises three phases of treatment: induction, consolidation and maintenance.
Some patients will be categorised as having a high risk disease (eg. Philadelphia chromosome positive (Ph+) ALL and Burkitt’s leukaemia). They will require special treatment programmes with different forms of medication and (perhaps) allogeneic bone marrow cell transplantation.
The induction phase consists of intensive chemotherapy including intrathecal medication and sometimes radiotherapy to the brain.
Approximately 80% of newly-diagnosed adults with ALL go into complete remission after the induction phase. This means that leukeamic cells are no longer found in the bone marrow, which returns to normal as do routine blood tests. Once remission is achieved, chemotherapy is continued at lower doses to destroy remaining leukaemic cells and so avoid relapse. Following the consolidation phase of treatment, maintenance treatment with a less intensive regimen of chemotherapy is required for a period of 2 or more years.
Patients who relapse can achieve a second remission using a second induction phase of treatment.
Acute Myeloid Leukaemia (AML)
Treatment of AML is usually divided into two phases: induction (to induce a remission) and consolidation (or post-remission) therapy.
The induction phase aims to destroy the majority of leukaemia cells so that there are no visible signs of the disease. This is achieved using intensive chemotherapy - usually given in hospital and lasting for about 1 week. However, the exact treatment protocol varies from patient to patient and hospital to hospital. It may include the use of intrathecal medication if the leukaemia has spread to the brain or spinal cord.
If this treatment fails to induce a remission, it can be repeated.
Following the induction phase, more chemotherapy is provided in the consolidation phase to destroy remaining cells. This may include several courses of high-dose chemotherapy (using different medication) or an allogeneic or autologous bone marrow transplant.
Stem cell transplants are intensive treatments with significant risks of serious complications but many specialists believe that an allogeneic transplant offers the best chance for survival.
Chronic Lymphatic Leukaemia (CLL)
There are a number of treatment options for people with CLL. Recommended treatment varies according to the stage of the disease and any symptoms it is causing. In many cases, clinical management is confined to monitoring the condition until it starts to progress or symptoms appear, when treatment is started.
In low-risk CLL, the prognosis is good and many patients can simply be observed. The treatment given is usually chemotherapy when required.
Those with intermediate and high risk CLL may still not need treatment immediately if the condition is asymptomatic but rather they can be carefully observed.
When treatment is required there are several options: fludarabine is used particularly in younger people - sometimes given with cyclophosphamide, chlorambucil or a monoclonal antibody (rituximab) or with a combination of all three.
Radiotherapy is rarely used but symptoms of CLL might include an enlarged spleen or swollen lymph nodes which will shrink rapidly with radiotherapy. Splenectomy is another option.
Chronic Myeloid Leukaemia (CML)
Treatment for patients with CML depends on which phase of their disease they are in and to some extent their age, as well as the availability of a suitable stem cell donor with matching tissue type.
In the chronic phase, treatment with imatinib is usual. Imatinib is a monoclonal antibody which works by blocking an abnormal enzyme found in CML.
For patients who have entered the blast phase, a stem cell (bone marrow) transplant is the best treatment, but there is no guarantee that it will work. Before a transplant, high dose chemotherapy and/or radiotherapy is used to destroy the patient’s existing bone marrow. Transplants work best in the chronic stage of the disease.
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Prognosis
Many factors affect prognosis including the stage of the leukaemia: the patient’s age, general health and their individual response to treatment (which will vary). For this reason, statistics can only be considered as a general guide as they are produced by grouping together patients in whom these factors are similar but not identical. These figures are historical, often covering a 10 or 20 year period, and so do not reflect improvements in survival through more modern treatment.
Survival rates indicate the percentage of people who survive the disease for a specific period of time after their diagnosis – usually 5 or 10 years.
Acute Lymphoblastic Leukaemia (ALL)
Children: as many as 85% of children with ALL will be cured.
Adults: 50% of adults will survive for 5 years or more.
Chronic Lymphocytic Leukaemia (CLL)
75% of patients survive for 5 years or more.
Hairy Cell Leukaemia (HCL) a form of CLL
Over 95% survive 10 years or more.
Acute Myeloid Leukaemia (AML)
40% of patients survive for 5 years or more.
Chronic Myeloid Leukaemia (CML)
90% of patients survive for 5 years or more.
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Key Trends
The main recent development has been the introduction of tyrosine kinase inhibitors, notably in the treatment of chronic myeloid leukaemia (imatinib).
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Important Research Areas
- Further use of tyrosine kinase inhibitors in the treatment of the other leukaemias.
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