chronic myeloid leukemia | 7 questions about chronic myeloid leukemia

7 questions about chronic myeloid leukemia




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World Chronic Myelogenous Leukemia Awareness
The healed patient had cancer that usually responds to immune-boosting therapies, but often ends up defeating them and is rarely cured. The healed patient was diagnosed with cancer usually responding to immune-boosting therapies, but often ends up defeating them and is rarely cured. JGT / SCIENCE


1. What is "chronic myeloid leukemia"?
Chronic myeloid leukemia (CML) "is characterized by excessive and persistent production within the bone marrow of white blood cells," explains the French Society of Hematology (SFH). Some of these white blood cells are abnormal because their development is not complete (we speak of "immature" cells) when they pass into the blood.

2. What is the origin of this disease?
The disease is linked to the appearance of an anomaly due to the fusion of two chromosomes (on the 9th and 22nd) of bone marrow stem cells, causing the appearance of a small abnormal chromosome, the Philadelphia chromosome (from name of the city where the two researchers who discovered it in the 60's worked). The presence of the chromosome induces the production of a gene (named "BCR-ABL") that codes for the synthesis of an enzyme (tyrosine kinase). But this enzyme is responsible for the production of white blood cells. Synthesized in excess, it produces too many white blood cells.

MYELOID. Leukemia refers to a disease of the blood, which is characterized in particular by a significant increase in the number of white blood cells. The term "myeloid" refers to the bone marrow, the tissue contained in the bones where all the cells of the blood are produced. We talk about a chronic disease when it settles gradually and slowly evolves at first.

3. Is it hereditary?
"The Philadelphia chromosome is a genetic abnormality acquired by abnormal stem cells, so it's not hereditary," says SFH. The causes of its appearance are so far unknown. However, there has been a higher incidence of CML among survivors of the atomic bombings in Hiroshima and Nagasaki. Ionizing radiation has therefore been suspected of being able to cause the disease, but this has never been formally demonstrated.

4. What are the symptoms?
CML evolves slowly at first and does not result in any particular symptoms, apart from a moderate fatigue and an increase in the size of the spleen. It is thus usually discovered fortuitously, during a blood test that shows an increase in the number of white blood cells.

5. How common in the population?
CML is a relatively rare disease, since there are about 600 new cases per year in France, according to the SFH. It occurs more in men than in women and its frequency increases with time: the median age of patients at the time of diagnosis is 53 years.6. How is the diagnosis made?
The diagnosis is based on several examinations, including bone marrow (myelogram) and blood tests to detect the BCR-ABL gene.

7. What are the treatments?
The treatments, to be taken continuously, are based on the administration of drugs that prevent the tyrosine kinase enzyme from functioning (these are the "tyrosine kinase inhibitors"). The number of white blood cells decreases and therefore becomes normal again. "These treatments are usually associated with a good therapeutic response, says the SFH treated people lead a life almost normal." In September 2015, a team of French researchers and doctors published in the journal Nature a study showing that it is possible to destroy tumor stem cells, resistant to anticancer treatments, of chronic myeloid leukemia in the elderly.