Chronic lymphocytic leukemia

A chronic lymphocytic leukemia (CLL) is a malignant disease of the lymphatic system, which is accompanied by an increased synthesis of non-functional lymphocytes. With over 30 percent, chronic lymphocytic leukemia is the most common form of leukemia in adulthood, especially from the age of 70.

What is Chronic Lymphocytic Leukemia?

Chronic lymphocytic leukemia (CLL) is a low-grade disease of the lymphatic system (B-cell non-Hodgkin lymphoma) with a leukemic course that can be traced back to clonal cell proliferation of the lymphocytes (white blood cells).

Chronic lymphocytic leukemia manifests itself in the form of swelling of the lymph nodes, enlargement of the spleen and liver, tiredness and weakness, anemia, thrombopenia (low blood platelet count) and specific skin changes (itching, eczema, mycoses, nodular infiltrates, skin bleeding as a result of increased bleeding tendency Herpes zoster) and a generally increased susceptibility to infections (recurrent pneumonia, bronchitis).

In addition, parotitis (Mikulicz syndrome), leukocytosis in combination with lymphocytosis, antibody deficiency syndrome, incomplete warmth autoantibodies, a reduced antibody concentration with a simultaneous increased concentration of lymphocytes in the bone marrow belong to the clinical picture of chronic lymphocytic leukemia.

causes

Chronic lymphocytic leukemia is due to the clonal proliferation of small cell and functionless B-lymphocytes. The exact causes for the increased synthesis of B-lymphocytes have not yet been clarified.

It is certain that CLL is usually (80 percent) caused by acquired genetic changes in the chromosomes, with the majority of cases being a deletion (lack of a chromosome sequence) on chromosome 13. Missing sequences on chromosomes 11 and 17 and a trisomy 12 (triple presence of chromosome 12) can also cause CLL.

The triggers for these chromosomal changes are not known, but bacterial, viral or parasitic infections are excluded. In addition, chemicals (especially organic solvents) and a genetic predisposition are discussed as triggers.

Symptoms, ailments & signs

The symptoms of this disease have a very negative effect on the quality of life and can significantly limit and complicate the everyday life of the person concerned. Primarily, the patient's lymph nodes are swollen and can also be painful. There is also an enlargement of the liver and spleen, which is also associated with pain.

If the disease is not treated, in the worst case it can lead to liver insufficiency and thus to death of the person concerned. A rash and severe itching appear on the skin. Bleeding can also occur on the skin and significantly reduce the aesthetics of the patient.

In many cases the disease leads to anemia and thus to severe tiredness and exhaustion of the person concerned. The patients therefore no longer take an active part in everyday life and need help from other people in their everyday life. It can also lead to nosebleeds and bruises in different parts of the body.

If the disease is left untreated, the patient's life expectancy is significantly reduced. As the disease progresses, it also leads to psychological upset or depression.

Diagnosis & course

An initial suspicion of chronic lymphocytic leukemia arises from the characteristic symptoms (including swelling of the lymph nodes on the neck, armpits, groin).

The diagnosis is confirmed by a complete blood count or differential blood count as well as an immunophenotyping of the lymphocytes. If an increased number of lymphocytes in the serum (over 5000 / µl) can be determined for at least 4 weeks and if the lymphocytes characteristic of CLL (deviating surface proteins, Gumprecht's umbra) can be detected in the blood smear, chronic lymphocytic leukemia can be assumed.

Imaging methods such as sonography and computed tomography provide information about the involvement of the internal organs (enlargement of the spleen, liver) and the severity of the disease. The course of CLL is heterogeneous and depends on the underlying chromosome change.

For example, those affected with a deletion on chromosome 13 have a comparatively favorable prognosis with a slow course, while chronic lymphocytic leukemia as a result of a deletion on chromosome 17 and 11 usually has a severe course with an unfavorable prognosis.

Complications

Chronic lymphocytic leukemia can lead to various complications. A very common negative side effect of this disease is the so-called antibody deficiency syndrome. The CLL cells, which are characteristic of lymphatic leukemia, displace the functional B cells in the human immune system.

As a result, the risk of infection increases immensely. At the same time, the body's defense against pathogenic germs is weakened due to the lack of functioning B cells. In some cases the amount of granulocytes is decreased. The body needs these to protect itself from bacteria. Those affected develop bacterial infections more often.

The respiratory tract and the organs of the gastrointestinal tract are particularly often affected. Infestation of the lungs with viruses or bacteria, in particular, can have serious effects without adequate timely treatment. In the worst case, lung infestation can be fatal. In addition to an antibody deficiency system, autoimmune hemolytic anemia can develop.

The result is paleness, tiredness, shortness of breath and ringing in the ears. Later on, further complications such as fever, chills, abdominal pain and vomiting can occur. In particularly severe cases, this condition can lead to kidney failure or shock.

Another serious complication is the development of malignant lymphoma. This transition is also known as the Richter transformation. It has a much worse prognosis than chronic lymphocytic leukemia.

When should you go to the doctor?

Since chronic lymphocytic leukemia is a malignant disease, a doctor should be consulted immediately if there is a persistent feeling of illness or a vague suspicion of a disagreement. The course of the disease is insidiously progressive and can be fatal without timely medical care. Due to the slow progression of leukemia, it is difficult for those affected to estimate when the symptoms will be triggered by a serious illness.

People who have been unusually tired for a long time or who have pale faces for several weeks should already use these tips and consult a doctor. Swollen lymph nodes in different areas of the body are considered worrying and need to be clarified by a doctor as soon as they can be felt for several weeks. This is especially true if the painless swellings have not developed as a result of flu.

A general weakness or states of exhaustion with adequate sleep and without mental or emotional stress should be clarified by a doctor. Powerlessness that arises for incomprehensible reasons must be examined and treated as quickly as possible. In the event of perceived anemia, a doctor's visit is also necessary. If the fingers or toes cool down unusually quickly or are permanently cold despite normal temperature conditions, it is advisable to consult a doctor.

Doctors & therapists in your area

Treatment & Therapy

In chronic lymphocytic leukemia, the choice of therapeutic measures correlates with the stage of the disease. In the early stages of CLL (Binet A and B), if there are no symptoms, no therapeutic measures are usually required.

On the other hand, in the case of advanced chronic lymphocytic leukemia (Binet C) or unfavorable chromosome changes, an early start of therapy is indicated. The treatment options here primarily include chemotherapy, in which the cell division of cancer cells is inhibited with drugs and the number of lymphocytes in the bone marrow is reduced, and immunotherapy in which the cancer cells are killed by genetically synthesized immunoglobulins.

For chemotherapy, the alkylants chlorambucil and bendamustine are used in combination with rituximab, the purine analogues deoxycoformycin, chlorodeoxyadenosine or fludarabine, whereby a combination of cyclophosphamide, fludarabine and rituximab is usually recommended for physically fit sufferers. Alemtuzumad, an anti-CD52 antibody, synthesized as part of immunotherapy is used monotherapy.

Since chronic lymphocytic leukemia is a systemic disease, radiation therapy is only possible locally in the case of large lymphomas, while bone marrow or stem cell transplants are only performed in cases of refractory (unaffected) or early relapsed CLL due to the high mortality rate.

In addition, supportive measures (replacement of blood cells with transfused erythrocyte or platelet concentrates, antibiotics) in chronic lymphocytic leukemia are indicated to prevent complications.

Outlook & forecast

Patients with chronic lymphocytic leukemia can often suffer from the disease without symptoms for several years. The leukemia can have a benign course for up to 20 years without any adverse effects on health. This leads to a creeping spread of the blood cancer in the organism.

The disease often develops over a long period of time with only a slight increase in symptoms. Few patients have a rapid disease course in which, within a few months of the onset of leukemia, the lymphocytes increase sufficiently that treatment is initiated. For this reason it is almost always discovered at a very late stage of development and only then can be treated.

However, the time of diagnosis is decisive for the healing process. The later CLL is discovered, the worse the prognosis. In addition, older people over 70 years of age often develop chronic lymphocytic leukemia.Due to their old age, the patients usually have other diseases that lead to a general weakening of the immune system and a reduction in the chances of recovery. If the lymphocytes are already in the bone marrow or if the liver or spleen has already enlarged, the chances of recovery worsen considerably.

prevention

Since the causes of the chromosome changes underlying chronic lymphocytic leukemia have not yet been clarified, the disease cannot be prevented. However, organic solvents and familial prevalence (disease frequency) are considered risk factors for the manifestation of CLL.

Aftercare

Chronic lymphocytic leukemia requires follow-up care if the patient has been discharged symptom-free. Otherwise, he must continue to be treated until he is symptom-free. Symptom-free patients with chronic lymphocytic leukemia should have their blood cells checked by the treating doctor every three to six months. The reason for the close monitoring is possible relapses.

In order to track and document the treatment effect, physical examinations for typical signs of a relapse are useful during follow-up care for those affected. Blood and bone marrow tests are also done in chronic lymphocytic leukemia. If in doubt, imaging methods such as ultrasound and computed tomography can also provide information about the current state of affairs. If there is a relapse, only blood stem cell transplants, cellular immunotherapies and kinase inhibitors can help.

A patient who agrees to participate in clinical trials of chronic lymphocytic leukemia during follow-up will be exposed to specific laboratory methods. For example, he will experience an immunophenotyping or an examination for the polymerase chain reaction. This can detect even the smallest amounts of degenerated blood cells in the organism.

Decisive for the scope and intensity of the follow-up measures is also which other illnesses the person concerned had previously suffered from. If the general condition is good, the follow-up measures can be carried out less often than in the case of previous illnesses that impair the general condition.

You can do that yourself

Chronic lymphocytic leukemia usually does not cause any symptoms for a long time, so patients can go about their everyday activities as usual. Sports activities are still possible, but the individual performance limit must not be exceeded. Because of the increased risk of infection, those affected should avoid traveling to tropical areas. Vaccinations with a live vaccine can have a negative effect on the course of the disease, but annual influenza vaccination with a dead vaccine is recommended.

Regular exercise, preferably in the fresh air, and a healthy diet rich in vitamins strengthen the immune system and reduce susceptibility to infections. Especially in the cold season, crowds should be avoided and special emphasis should be placed on regular and thorough hand washing. Careful washing of fruit and vegetables can significantly reduce the number of germs ingested, and avoiding unpasteurized milk products reduces the risk of listeriosis infection.

If symptoms of an infectious disease such as fever, diarrhea or breathing difficulties occur despite the precautionary measures, immediate medical treatment is urgently recommended. During and after chemotherapeutic treatment, protection against infections and their rapid control is particularly important. Chronic lymphocytic leukemia can also affect the psyche: Exact information, talking to family and friends or talking to people in a self-help group can help you to accept the disease and to cope better with it.