Chronic Lymphocytic Leukemia Course, Symptoms & Causes

Chronic Lymphocytic Leukemia (CLL)

Chronic lymphocytic leukemia, abbreviated to CLL, is a lymphoma that develops from what are known as B cells.

Non-Hodgkin’s lymphoma (NHL) refers to all those malignant that are not Hodgkin’s disease. This applies to a large number of very different diseases , which can also have completely different causes and courses.

Chronic lymphocytic leukemia originates from B lymphocytes (B cells). These cells belong to the white blood cells ( leukocytes ) and are therefore one of the essential components of the immune system. B lymphocytes are responsible for the formation of antibodies and are located in the bone marrow.

In the case of chronic lymphocytic leukemia, the B cells are mature but have degenerated and are therefore non-functional. The causes probably lie in a genetic change, which can vary in severity.

Because the functionless, degenerated B cells multiply and are also detectable in high amounts in the blood, this type of lymphoma is referred to as a leukemic form (blood cancer). Due to the spread of the degenerated cells in the blood, it is also a form of blood cancer .

The course of CLL in individual patients can be very different. This essentially corresponds to the severity of the genetic change in the B cells.

Chronic lymphocytic leukemia course

The causes of the disease have not yet been fully clarified, and there is no evidence that viruses are involved. The cause is likely to be genetic changes, through which the B lymphocytes mature but become inoperable. In the vast majority of cases, the disease is only discovered at an advanced age (> 65 years); men are usually more than 1.5 times more likely to be affected than women.

The course of chronic lymphocytic leukemia is typically very mild and progresses slowly, many sufferers do not need treatment for many years. However, the severity of the disease and the course depend essentially on the type of chromosomal change.

In most cases there is a deletion on chromosome 13 . This means that the course of the disease is relatively mild. In this case, part of chromosome 13 is missing from the genetic code of B lymphocytes.

Other possible changes affect chromosomes 11, 12 and 17. If there is a deletion on chromosome 17, the course is more severe and the prognosis is less favourable.

Classification of stages in the course of chronic lymphocytic leukemia

The disease is divided into different stages as it progresses. The stages are denoted by the letters A, B and C. In addition, the stages are divided by numbers in order to be able to assess the progress of the disease more precisely.

According to this classification, in most cases there is no need for treatment until stage C has been reached. Depending on the course, this stage can be reached either very quickly or over a period of years.

Chronic lymphocytic leukemia progression stage A

At this stage, 3 or fewer lymph node regions are affected, but the lymphocyte count in the blood count is already increased (at least 5,000/µl)

Stage A0: here no lymph nodes are enlarged
Stage AI: here there are already individual, palpably enlarged lymph nodes
Stage A II: in addition to the enlargements, there is already an enlargement of the liver and/or spleen

Chronic lymphocytic leukemia course stage B

At this stage, more than 3 lymph node regions are affected

Stage BI: there is an infestation, but no enlarged nodes are palpable
Stage B II: there is an enlargement of the liver and/or spleen

Chronic lymphocytic leukemia course stage C

At this stage, serious disorders in the blood count are already evident. There is a need for treatment. The quality of life is already severely reduced because of the anemia and the severe thrombocytopenia.

Stage C III: Severe anemia occurs with hemoglobin levels below 10 g/dl

Stage C IV: there is a massive platelet count (significant decrease in the number of platelets in the blood) with platelet counts of less than 100/nl

Anemia (anaemia) leads to an undersupply of oxygen to the entire organism, and the low platelet counts at this stage primarily lead to an increased tendency to bleed.

Both clinical pictures each have a large number of different symptoms. The treatment aims primarily at the therapy of these two serious clinical pictures.

In individual cases, chronic lymphocytic leukemia can also progress very quickly. In this case, the treatment sometimes has to start earlier.

Chronic Lymphocytic Leukemia Symptoms

At the beginning of the disease, many of those affected are asymptomatic, i.e. there are no symptoms to be noticed. In many cases, the diagnosis is made solely on the basis of a blood test and not on the basis of any particular symptoms.

The enlargements and swellings in the lymph nodes are painless, especially at the beginning, and usually only slightly disturbing. However, the enlargement of the liver and spleen can make itself felt in some of those affected by an uncomfortable feeling of pressure in the upper abdominal region. In many cases, this causes only a few problems and the well-being of those affected is usually hardly affected. Around a fifth of cases are completely symptom-free up to the advanced stages.

In addition to these symptoms typical of the disease, chronic lymphocytic leukemia can also cause symptoms that are also common in other leukemic or cancerous diseases:

• profuse sweating at night
• unintentional significant loss of face
• Bouts of fever (without an existing infection)

These symptoms are often referred to as “B symptoms”.

In most cases, no further symptoms are seen until anemia or severe thrombocytopenia occurs. In most cases, the general condition is hardly affected.

As the disease progresses, however, other chronic lymphatic leukemia symptoms appear, which are associated with the developing anemia, thrombocytopenia and the increasingly poorly functioning immune system.

Chronic Lymphocytic Leukemia Symptoms of Anemia

With the onset of anemia and due to the low hemoglobin (Hb) levels in the blood, the blood’s ability to transport oxygen is severely restricted. This affects many areas of the body:

• accelerated heartbeat (tachycardia) to compensate for the low oxygen transport capacity
• quick fatigue
• exhaustion
• Shortness of breath on exertion
• frequent headaches
• Tinnitus (not in all cases)
• pale skin and mucous membranes
• whitish sclera hair loss
• brittle nails,
• frequent nausea
• Syncope (fainting spells)
• Chest pain and heart ache (angina pectoris)
• in the further course often renal insufficiency

Chronic lymphocytic leukemia symptoms of thrombocytopenia

• Spontaneous bleeding, especially nosebleeds
• Tendency to hematomas and petechiae (bleeding under the skin)
• Gastrointestinal bleeding possible
• cerebral hemorrhage possible

Chronic Lymphocytic Leukemia – Symptoms caused by the compromised immune system

There can also be other symptoms that are primarily related to the impairment of the immune system by the no longer functioning B lymphocytes, such as an increased tendency to infection.

In about one third of the cases, there are also unpleasant skin symptoms such as itching, eczema or fungal diseases of the skin. Herpes zoster can also occur. Some of these symptoms can also be observed in the early stages of chronic lymphocytic leukemia.

CLL and the proliferation of lymphocytes

Chronic lymphocytic leukemia leads to a sharp increase in the number of white blood cells in the body. It is the most common type of leukemia in adults. It occurs predominantly in older people with an average age of 60 to 70 years.

Chronic lymphocytic leukemia (CLL) – disease of the lymphatic organ system

The presented cancer is considered to be a malignant disease affecting the lymphatic system. The latter consists of the lymph nodes, the lymphocytes and the lymph vessels, the lymph and the lymphatic organs.

The system is closely related to the immune system and the hematopoietic system.

The lymphatic system has the lymphocytes as cells. They belong to the white blood cells and perform important tasks in the immune system. If there is chronic lymphocytic leukemia – abbreviated to “CLL” – errors occur in the formation of the lymphocytes. The body either produces too many of these white blood cells or the lymphocytes do not mature.

Symptoms of CLL: swollen organs, tiredness and increased tendency to bleed

Visually, the lymphocytes do not change and retain their normal appearance. Nevertheless, the disease reduces the defense functions of the cells. The dysfunctional lymphocytes are located in the bone marrow, spleen and lymph nodes.

As a result of the malignant disease, the following lymphatic organs swell:

• the spleen,
• the liver,
• the lymph nodes in the neck, in the groin and under the armpits.

The more the diseased white blood cells multiply, the more space they take up in the bone marrow. There they displace the healthy components of the blood. As a result, there is a risk of anemia because there are no longer enough red blood cells to transport oxygen. In this case, the patient notices symptoms of weakness and fatigue. Too little production of platelets increases the tendency to bleed.

Diagnosis of chronic lymphocytic leukemia

Diagnosing CLL by examining the proteins on the lymphocytes

Despite the symptoms listed, chronic lymphocytic leukemia progresses very slowly in most cases. In the first few years, there are often no symptoms. Therefore, doctors often discover the disease during a routine examination. An indicator, for example, is a too high value of the white blood cells in the blood count.

If CLL is suspected, the doctor will carry out more detailed examinations to prove the diagnosis. The most reliable method is the immunophenotyping of the lymphocytes. The method identifies which proteins occur on the surface of the lymphocytes. The proteins in CLL differ significantly from the surface structure of healthy cells. If necessary, the doctor also decides on a bone marrow puncture to obtain final security.

Chronic lymphocytic leukemia usually affects the entire lymphatic system. After the diagnosis, it is therefore advisable to examine all the components of the system. The doctor scans all superficial stations of the lymph nodes. In addition, an examination of the internal organs using ultrasound follows. Computed tomography is also recommended.

Depending on the results of his examination, the doctor determines the current stage of progression of the CLL. There are two accepted classification systems called “Binet” and “Rai”. The classification is based on the restriction of blood formation and the symptoms in the lymphatic system.

Treatment of chronic lymphocytic leukemia

Treatment after symptoms appear

Since the presented form of leukemia progresses slowly in most patients, it initially causes no symptoms. There is often no need for treatment in the early years of the disease. The doctor only takes appropriate measures when symptoms such as overly large lymph nodes occur. If the leukemia progresses rapidly, the situation is different. In this case, treatment begins shortly after diagnosis.

There are two different options for treating this type of leukemia: immunotherapy and chemotherapy . Chemotherapy aims to kill diseased cells with drugs. However, the therapy has the same effect on the healthy cells. This leads to side effects such as nausea, vomiting or hair loss.

In immunotherapy, the patient receives manufactured proteins that attack the cancer cells. These are genetically engineered antibodies that the patient receives in weekly infusions. In most cases, the therapy leads to a reduction in the number of lymphocytes. A combination with chemotherapy increases the effect.

In summary, the presented chronic lymphocytic leukemia occurs predominantly in older people. Since the malignant cancer usually progresses slowly, it does not reduce the average life expectancy. There is no need for treatment until symptoms appear in the lymphatic system.