cll | Chronic lymphocytic leukemia

Chronic lymphocytic leukemia

Chronic lymphocytic leukemia is the most frequent of adult leukemias, with about 4 000 new cases per year in France. It affects men twice as many times as women. This disease occurs in the vast majority of cases after 50 years and is most often slow-changing.

The disease
Chronic lymphoid leukemia (LLC) is a disease affecting blood cells called B lymphocytes. These cells are produced by the bone marrow. They play an important role in the immune system, that is to say, all the mechanisms for defending against external agents (bacteria, viruses, fungi...). Like all cells, B lymphocytes normally have a limited lifespan, in the order of a few days to a few months. After this time, they die and are replaced by new cells, the production of lymphocytes being continuous. During the LLC, part of the B lymphocytes no longer dies. Their life cycle is somehow blocked when they reach maturity. As their production continues, these B lymphocytes eventually accumulate in the blood, in the lymph nodes, spleen and bone marrow, which explains the increased volume of the lymph nodes and spleen.

What are the signs of the disease?
At first, people with LLC generally feel no signs of the disease. This one goes completely unnoticed. It is common for the LLC to be diagnosed only several years after its onset. In fact, the main manifestations of the LLC are observed in the blood, with too many B lymphocytes. Abnormal results for other blood components (including hemoglobin and platelets) may also be observed, but they are not constantly found. It is when the disease evolves that some physical signs can be felt. It is possible that lymph nodes are larger than in ordinary times. Signs such as fatigue, fever or shortness of breath may occur during the LLC.

The diagnosis
The LLC is a disease affecting blood cells. His diagnosis is therefore based on three tests carried out on the basis of a simple blood test.

The number of lymphocytes in the first time, one carries out what is called a CBC or a blood formula count. It is a very common examination that consists of counting the different types of blood cells. The diagnosis of LLC can be evoked if the number of B lymphocytes is greater than normal values. A transient increase in blood cells is always possible (as a result of a viral infection for example), lymphocyte counts should be renewed for confirmation of the anomaly.
The characteristics of lymphocytes to be formally established, the diagnosis of the LLC requires the study of lymphocytes under a microscope. In the case of LLC, the appearance of these cells does indeed have specific characteristics that identify them and eliminate other diseases associated with an abnormally high number of lymphocytes.
The immunophenotyping the determination of immunophénotype is to study specific markers (antigens) on the surface of lymphocytes. When present, these markers are in a way the "signature" of the disease. This review makes it possible to affirm the diagnosis of the LLC. The immunophenotyping is carried out by a specialized laboratory

The Clinical exam

When the results are known, the diagnosis is announced during a consultation. The doctor then carries out a complete clinical examination. It also looks for the presence of lymph nodes that have increased in volume and see if the spleen is perceptible to palpation.

The evolution of the disease

• Chronic Disease

Unlike other so-called acute leukemias that evolve rapidly, the LLC is a slow-changing disease. That's why doctors see it as a chronic disease.

• The different stages

Doctors classify the LLC according to three stages:

Stage A is a moderate increase in the number of B lymphocytes, with no other blood abnormalities, with no or very limited presence of large ganglia.

Stage B corresponds to an increase in the number of B lymphocytes, with no other blood abnormalities, but associated with the presence of large lymph nodes in several ganglion territories.

Stage C corresponds to an increase in the number of B lymphocytes associated with other blood abnormalities: anemia (lower hemoglobin) and/or a decrease in the number of blood platelets.

• "3 thirds" the LLC is often presented, in relation to its evolution, as a "three-thirds" disease. Indeed, one can schematically consider that there are three modes of evolution of the disease:

In a third of the cases, the disease is at a very low stage and will not evolve or very little. Patients will never need treatment.

In one-third of the cases, the disease is at an undeveloped stage, not requiring immediate treatment, but will evolve in the years to come and then have to be treated.

In a third of cases, the LLC is scalable at the time of diagnosis and requires a fairly rapid treatment

Lexicon

Antigen: molecule capable of triggering an immune reaction.

Ganglion: Anatomical structure playing a role in the defenses of the organism.

Hemoglobin: A protein in the red blood cells that ensures oxygen is transported in the blood.

CBC: Examination of counting the number of different cells present in the blood.

Bone marrow: Structure at the level of all the bones where the elements of the blood are made.

Numerals: see CBC.

Platelets: Blood components that play an essential role in coagulation. In case of a decrease in their number, we talk about thrombocytopenia and there is then a risk of bleeding

Your follow up

As with any chronic disease, the LLC requires regular medical follow-up, even if no treatment is taken. This medical follow-up must be maintained throughout life.

• Who to consult?

It is recommended that you contact a hematologist, a doctor who specializes in blood diseases. Your attending physician is able to direct you to the hematology Department of a hospital centre. The hematologist will ensure your management in coordination with your treating physician that you can, of course, continue to consult.

• Pace of consultation

If you do not receive any treatment for your LLC, it is advisable to consult your hematologist every six months to one year to determine if your illness is still stable. If you have started or already taken a treatment, the pace of your follow-up depends on your health and is set by your hematologist.

• Clinical Follow-up

During the consultations, your hematologist will examine you. It will also look for palpation if lymph nodes and spleen have increased in volume, and it will note their evolution since your previous visit. The consultation will also be an opportunity to make a point about your general health status.

• Biological monitoring

The first step is to monitor the evolution of CBC, especially the rate of B lymphocytes. The other biological parameters measured are mainly the number of platelets and the rate of hemoglobin, in order to detect a possible anomaly

The main possible complications

During the LLC, several types of complications may occur. These require appropriate management and there are treatments to control them.

• infections

The LLC can be accompanied by an immune deficiency, that is, a decrease in the body's defenses. In this case, the latter is less able to control viruses, bacteria and fungi. As a result, infections can become more frequent. They most often affect the respiratory system (nose, throat and lungs). There are effective treatments for these infectious episodes.

• Lower platelets

Platelets are elements of the blood that play an important role in the coagulation process. The LLC may cause a decrease in their rate (this is referred to as thrombocytopenia). When the drop is pronounced, there is a risk of bleeding (e.g. in the gums). Transfusion of platelet concentrates is possible to correct the reduction of these cells.

• anemia

The disease may result in anemia, that is, a decrease in hemoglobin levels in the blood, which results in fatigue. To treat anemia, it is possible to use red blood cell transfusions.