Chronic myelogenous leukemia (CML)


What is it about ?

Chronic myelogenous leukemia (CML) is a malignant disease of the bone marrow, more specifically of the white blood cells. Bone marrow is located inside the bones and is responsible for the production of red blood cells, white blood cells and platelets. In CML, the production of white blood cells increases, releasing large amounts of abnormal and immature white blood cells into the blood. The production of red blood cells and platelets is therefore threatened.

CML is a disease that progresses slowly; this is why it is called a ‘chronic disease’. The disease usually progresses in three stages. During the chronic phase, which can last for several years, the high number of white blood cells remains more or less stable. Then comes the acceleration phase, in which the number of white blood cells begins to increase more rapidly. Finally, in the final phase, the disease becomes aggressive and it is a question of acute leukemia.

What is its frequency?

Each year, this form of leukemia is diagnosed in 1 in 100,000 adults. It is the rarest form of leukemia, accounting for 20% of leukemia cases. The disease affects men as often as women. The majority of people who develop chronic myelogenous leukemia are between the ages of 40 and 70, with a peak occurring around the age of 55. The disease occurs very rarely in children.

How to recognize it?

The chronic phase is usually not accompanied by any symptoms. But, as the disease progresses, the following symptoms may appear: night sweats, high temperature, fatigue and weight loss. Since CML sometimes goes together with an enlargement of the spleen (enlarged spleen), pain may be felt under the ribs on the left side of the upper abdomen.

How is the diagnosis made?

The diagnosis is made by means of a blood test. If the test result is abnormal, the general practitioner will refer you to a specialist in blood and blood diseases (hematologist). The latter will take a little bone marrow via the breastbone or pelvis and analyze it.

We will also look for specific genetic abnormalities: a Philadelphia chromosome (a defective chromosome due to the exchange of pieces of genetic material between chromosomes 9 and 22) or a fusion gene in the bone marrow and / or blood.

What can you do ?

If you suffer from prolonged fatigue, night sweats or unexplained weight loss, it is advisable to see your GP.

What can your doctor do?

Treatment depends on what stage the disease is in at the time of diagnosis. In the chronic phase, the number of white blood cells can be reduced with medication. Immunotherapy is another possibility, involving taking tablets that inhibit the Philadelphia chromosome. In the acute phase, a stem cell transplant is sometimes necessary.

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Source

Foreign clinical practice guide ‘Chronic myelogenous leukemia (CML)’ (2000), updated on 13.06.2017 and adapted to the Belgian context on 14.07.2019 – ebpracticenet