Chronic Myeloid Leukaemia (CML)

Leukaemia is a cancer of the white blood cells.

White blood cells help to fight infection. There are two different types of white blood cell lymphoid cells (lymphocytes) and myeloid cells. CML affects the myeloid cells.

Normally the white blood cells, which are produced in the bone marrow, repair and reproduce themselves in an orderly and controlled way. In leukaemia, however, the process gets out of control and the cells continue to divide but do not mature.

These immature dividing cells – known as blast cells – fill up the bone marrow and stop it making healthy blood cells. As the blast cells are immature, they cannot work properly. This puts the child at increased risk of infection. The overproduction of white blood cells also interferes with the production of healthy red blood cells and platelets, leading to symptoms of Leukaemia such as anaemia and bruising.

In contrast to acute leukaemias (such as acute myeloid leukaemia and acute lymphoblastic leukaemia), this happens very slowly in CML

There are three different stages in the development of chronic myeloid leukaemia: 

1. Chronic phase. There may be no symptoms of leukaemia, but blast cells are present in the blood and bone marrow. This phase can last several years before undergoing transformation to the second, accelerated phase.
2. Accelerated phase. An increased number of blast cells are found in the blood and marrow and there is evidence that the number of normal cells is decreasing. This phase lasts three to nine months.
3. Blast phase. Also called a ‘blast crisis’. During the third and final phase, the disease resembles acute leukaemia. More than 30% of bone marrow and blood cells are blast cells. Without effective treatment (see below) the disease is usually fatal within three to six months of entering the blast phase.

With Chronic Myeloid Leukaemia (CML), there are usually no symptoms in the early stages. Often it may be discovered when the child is having a routine blood test for other reasons.
When symptoms of chronic myeloid leukaemia do occur, they are quite general, including:

• Frequent, persistent infections
• Unusual bleeding and bruising
• Tiredness
• Paleness
• Breathlessness

There may also be bone/joint pain, abdominal pain and/or swollen lymph nodes.

Children may have some or all of these leukaemia symptoms.

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Further tests will be carried out to provide doctors with as much information as possible about the child’s disease.

Unlike the acute leukaemias, childhood CML cannot be cured by any type of chemotherapy although a new drug called Imatinib, a type of tyrosine kinase inhibitor, or TKI, can eliminate easily detectable signs of leukaemia from the blood, prolonging the chronic phase of the disease and delaying transformation to the accelerated stage. Imatinib cannot cure chronic myeloid leukaemia. It must be taken every day to prevent progression of the disease.

The only treatment that can cure childhood CML is a stem cell transplant.

Five year survival from childhood CML has increased in recent years thanks to the introduction of new drugs (known as tyrosine kinase inhibitors, or TKIs) that prevent, or at least delay, progression from the chronic phase to the accelerated phase.

Currently more than 90% of children diagnosed with chronic myeloid leukaemia survive five years or more from diagnosis.

It is important to note, however, that these drugs do not cure CML. They have to be taken every day in order to prolong the chronic phase.