cml | Chronic Myelogenous Leukemia

Chronic myeloid leukemia

Your doctor has taught you that you have been reached
of "Chronic myeloid Leukemia" (CML).
The term "leukemia" means that it is a
A blood disease that is particularly characteristic
By a significant increase in the number of
White blood cells.
The term "myeloid" refers to the marrow
Bone, that is, the tissue contained in the
Bone where all blood cells are produced
(not to be confused with the spinal cord that
belongs to the nervous system). An anomaly
Chromosome of the stem cells of the marrow
(those that produce blood cells) is
The origin of the increase in white blood cells.
The term ' chronic ' means that the disease
Progressively settled and evolves
Slowly at first.
Chronic myeloid leukemia is part of the blood diseases grouped
Under the name "Myeloproliferative syndromes". It is characterized by a
Excessive and persistent production within the bone marrow of the blood cells
Whites (or leukocytes). Some of these white blood cells are abnormal; This
Are immature cells, that is, the development is not completed
When they pass into the blood.
The disease is linked to the appearance of an anomaly related to the fusion of two
Chromosomes of bone marrow stem cells, causing the onset of
of a small abnormal chromosome, the Philadelphia chromosome (from the name of the
City of the United States where the two researchers who discovered it were working in
The years 60). This anomaly results from the erroneous assembly of a gene from the
Chromosome 9, known as ABL, with a chromosome 22 gene, named BCR.
This produces the so-called BCR-ABL gene that is present only in the cells of
The disease. This gene produces abnormally an enzyme, tyrosine kinase, itself
Responsible for the increased production of white blood cells.
The Philadelphia chromosome is a genetic anomaly acquired by the cells
Abnormal strains; It is therefore not hereditary. The causes of its appearance
are so far unknown. However, there was a more frequent
Important part of the CML among the survivors of the atomic bombing
Hiroshima and Nagasaki. Ionizing radiation was therefore suspected of
Be able to provoke the disease, but this has never been formally demonstrated.
Chronic myeloid leukemia is a relatively rare disease, since
There are about 600 new cases a year in France. It is a little more
Common in men than in women. Its frequency increases with age. The
Time of diagnosis, the median age of patients is 53 years.

The diagnosis
Chronic myeloid leukemia is a disease that slowly evolves in a
First time and which does not result in any particular symptom (apart from
More often moderate fatigue and an increase in the size of the
Spleen). It is thus usually discovered in a fortuitous way, on the occasion of a
Blood balance that shows an elevation in the number of white blood cells.
The diagnosis is then based on several exams:
• A blood count.
• A bone marrow swab (myelogram). Performed under Anesthesia
Local, this test involves inserting a hollow needle into a bone. This is
The sternum (flat bone in the middle of the chest) or the part of the
Protruding from the hip. A small amount of marrow is then sucked, which
Then it is possible to make a karyotype, that is, a study of the chromosomes,
and search for the Philadelphia chromosome. The levy also allows
To quantify the abnormal white blood cells present in the bone marrow.
• Molecular biology blood tests aimed at detecting the gene
BCR-ABL, to measure the amount of cells that carries it (so-called
"BCR-ABL Load"), and thus quantify the number of leukemic cells.
The diagnosis is established when the Philadelphia chromosome and the BCRABL gene
are found. The various exams made also make it possible to
Determine the stage of the disease.

Chronic myeloid leukemia (cont'd)

Your useful contacts

• Secretariat/Appointment:

• Nurse Consultation:

• Psychologist Consultation:

• Social worker:

• In case of emergency:

The main side effects

of treatments

CML treatments can lead to

Side effects. The most common

are leg cramps,

As well as edema (swelling

of the skin), including the

Face, especially the eyelids. The

Swellings appear especially at the beginning of the

Treatment. A weight gain is also

Frequently reported, as well as

Digestive disorders (nausea, vomiting or

Diarrhea). These side effects are not

Systematic and their intensity is variable

From one person to another.

Federation leukemia Hope

Website: http://www.leucemie-espoir.org. Patient Association D

Any treatment is likely to induce effects

and may pose risks. Your

Doctor will inform you and indicate the signs

To be monitored before you start treatment

Proposed.

Participate in a clinical trial

The best way to advance the

The burden of a disease is to treat patients

In the context of therapeutic trials.

If your doctor proposes to participate in the

A clinical trial, he will explain the purpose,

The progress, the expected benefits, the

Potential risks, and will give you a notice

of information.

Participating in a trial requires that you give the

Prior written consent

The evolution of CML

Chronic myeloid leukemia evolves into three phases:

• The Chronic phase.

It is at this stage that the disease is diagnosed in most patients.

During this phase, leukemia evolves slowly and there is little or no

Symptoms. There are still few abnormal white blood cells in the marrow

Bone and in the blood. This phase lasts on average four in the absence of

Treatment.

• The acceleration phase.

It corresponds to an increase in the proportion of white blood cells

Abnormal in the blood and in the marrow, as well as an elevation of the

BCR-ABL load or the emergence of new chromosomal anomalies.

Non-specific symptoms are more common, such as fatigue, loss of

Appetite, fever for no apparent reason. If a treatment is not implemented,

The disease evolves after several months in the acute phase.

• The transformation phase.

of chronic, leukemia then becomes acute. The bone marrow is invaded

By the abnormal white blood cells and can no longer function properly. The

Illness is very serious.

The treatment

The therapeutic management of chronic myeloid leukemia is based on

The administration of drugs called tyrosine kinase inhibitors. These

Act by preventing an abnormal enzyme from functioning. By taking

Of one of these drugs, the number of white blood cells decreases and becomes

Progressively normal. At the same time, the amount of BCR-ABL in the blood

Drop and the Philadelphia chromosome ended up no longer being detected in the marrow

Bone.

This chronic phase treatment of the disease is usually associated with

A good therapeutic response. In the absence of efficiency or if it proves to be

is not sufficient, it is possible to replace the prescribed drug with another

tyrosine kinase inhibitor; Currently, several drugs of this type

are indeed available.

It is necessary to take the treatment on an ongoing basis, i.e. all the

Days without interruption.

The Monitoring

People under treatment who have normalized blood cells

Whites must consult their hematologist every three to four months. The

The purpose of the consultations is to check the general health status. They also give rise

To different blood tests, in particular a measure of the amount of BCRABL

In the blood. Sometimes a control of drug concentrations in the

Blood is necessary to ensure the correct dosage.

With current medications, people with myeloid leukemia

Chronic treatment can now lead a life almost

Normal.