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An aggressive cancer of the cells of the lymphatic system is large cell lymphoma. The disease is characterized by uncontrolled growth and division of cells, but the natural death of old cells does not occur during this process. A malignant lesion can occur in one or more lymph nodes, and in a progressive form, the pathology spreads to the vital internal organs of a person.
What is the essence of the disease?
B-cell lymphoma is difficult to treat
At the moment, experts have not established the exact causes of the development of b cell lymphoma. Therefore, in the ICD-10 classification it is assigned code C85.1 with the label “unspecified B-cell lymphoma.”
A feature of the disease is its origin from beta cells or b-lymphocytes. The main function of these cells is to maintain humoral immunity. Through their activity in the body, an immunomodulatory function is ensured.
When a pathological process occurs in the structure of beta cells, the body’s defense system fails, as a result of which it becomes unprotected against the penetration of parasites, bacteria, fungi and other pathogenic microflora. As a result, the patient may develop numerous diseases.
Many modern studies point to the fact that today the development of large cell lymphoma is so rapid that it has reached epidemic proportions. The main reason for such a high incidence of the disease is considered to be the increasing incidence of immunodeficiency.
Until recently, the prognosis for this disease was disappointing. But with the advent of cytostatics, the chances of recovery have increased significantly.
Risk group
Non-Hodgkin's lymphomas are being diagnosed more and more often. The neoplasm can occur in people of any age. More often diagnosed in elderly patients. The peak incidence occurs at 65 years of age.
This lymphoma can affect any internal organs. The aggressive nature of the pathology causes failure of vital systems. Without treatment, the chance of survival is zero. Chemotherapy can cure the disease and put a person into stable remission, increasing life expectancy.
Hodgkin lymphoma consists of macrophage and monocytic cells. The disease is lymphogranulomatosis. Cellular composition is the main difference between the disease and non-Hodgkin pathologies.
Histology of Hodgkin's lymphoma
Causes
Since at the moment there are no reliable reasons for the development of lymphoma b, doctors have to be content with hypotheses and approximate data. In many countries around the world, studies are being conducted to determine the relationship between the development of lymphoma and exposure to carcinogenic and toxic substances on the human body.
Possible causes of the development of beta-cell lymphoma are:
- hereditary predisposition;
- acquired immunodeficiency syndrome (AIDS);
- autoimmune diseases, including Crohn's disease, diabetes, multiple sclerosis, Sjögren's disease, etc.;
- consequences of hepatitis C and herpes;
- consequences of kidney and stem cell transplants.
In addition, hypotheses regarding the influence of external factors on the development of the disease are widespread in scientific circles:
- Epstein-Barr virus and diseases of similar etiology;
- exposure to carcinogenic substances, such as benzene, benzopyrene, formaldehyde, dioxins, etc.
One of the main reasons for the development of the disease is weakened immunity.
All of the factors described above in one way or another affect the structure of leukocytes, contributing to their uncontrolled division. The result is B-cell lymphoma.
Symptoms
When B-cell lymphoma affects the bone marrow, a person experiences severe headaches and dizziness
The manifestations of this disease are in many ways similar to the symptoms of other malignant neoplasms. The following signs indicate the development of large cell lymphoma in the body:
- weight loss that occurs suddenly and for no apparent reason;
- increased sweating at night;
- development of general malaise;
- increased body temperature;
- anemia and thrombocytopenia, causing increased bleeding and pale skin;
- rapid fatigue, and we are not talking about serious physical exertion;
- enlarged lymph nodes in groups.
In some cases, the pathological process spreads to nearby organs and bone apparatus. In this case, pain appears, which is accompanied by severe symptoms:
- If the respiratory system is damaged, the patient experiences a feeling of lack of air and coughing.
- If the pathology affects the bone marrow, then frequent dizziness, blurred vision and headaches occur.
- Damage to the intestines provokes the development of digestive problems and the urge to vomit.
Classification
Depending on the type and nature of the disease, there are three types of large cell lymphoma:
- With a low degree of malignancy. Seals form in the lymph nodes, and the nodes themselves do not exceed three centimeters in size. In this case, the patient does not have any complaints.
- Average degree of malignancy. In the area of the lymph nodes there are two or more hardenings with a pronounced bumpy surface, the size of which usually does not reach 5 cm.
- High degree of malignancy. The most severe form of the disease, in which a large number of nodes appear, measuring 3-5 cm. The neoplasms hurt and itch.
Regarding the nature of the disease, the following types of lymphomas are distinguished:
- Marginal zone. The tumor is characterized by pain at the site of node formation. They often appear in the abdominal cavity. In most cases, other symptoms are not observed.
With large cell marginal zone lymphoma, the patient experiences a rapid feeling of satiety while maintaining appetite.
- Follicular form. It involves the growth of lymph nodes in the armpit, groin and neck area. The development of pathology is accompanied by an increase in body temperature and the presence of pain in the places where the nodes appear.
- Diffuse lymphoma. The main symptom of this disease is the presence of skin rashes in the form of ulcers and plaques.
- Anaplastic large cell lymphoma. Enlarged lymph nodes occur in the neck area, as well as in the armpit area. The tumor is considered one of the most aggressive.
With the development of any of the above forms of the disease, the general symptoms remain, to which specific signs are added.
The most common subtype of B-cell lymphoma is large, diffuse.
Types of large cell lymphoma
There are several types of large cell lymphoma. It is worth considering only those that are most widespread.
Mediastinal form
This type of neoplasm can affect any organ in the thoracic region. The origin of the disease is associated with abnormal cell division and degeneration. Mutating cells form tissues that form cancerous tumors. They tend to grow quite rapidly and are aggressive in nature. By metastasizing, the process spreads to nearby organs and systems, disrupting their activity in the early stages.
Anaplastic form
In this case, the formation of lymphoma involves white blood cells, which must fight infection.
This form represents only 2% of all lymphomas, but 12% occur in children. Therefore, the greatest risk of occurrence is in younger people, under 30 years of age. Moreover, men are more susceptible.
Progression occurs quite quickly even in the initial stages. But more than obvious signs of the disease contribute to the detection of a tumor in the initial stages.
Damage occurs not only in the lymphatic system, it also affects the skin, lungs, liver, and soft tissues. Often the lesions are localized in the facial part, which leads to deformities and irreparable pathologies.
Treatment is quite long and relapses often occur.
Marginal form
The name of the form indicates exactly the location of the tumor. The marginal zone is located in the spleen, where there is a border between the red and white pulp, the concentration of a large number of lymphocytes and macrophages. This species is classified as sluggish.
The greatest symptoms in this case come from the digestive system. Attacks similar to pancreatitis occur, which can confuse an inexperienced doctor. But the primary symptoms, inflammation of the lymph nodes, show clinical carotene.
The main symptoms of marginal lymphoma are pain in the abdomen and the formation of nodes in the digestive system. When eating food, satiety occurs quickly. In this case, the disease is generally asymptomatic.
From cells of the mantle zone
This form is characterized by the rapid growth of cancer cells and the formation of chromosomal mutations. The symptoms are hidden and detection occurs at advanced stages of the disease. Older men are most susceptible to it. Risk factors and causes of the disease are determined individually, but basically they are identical to the others. Refers to slowly developing, but at the same time progressing. Although, on the contrary, it can develop quickly and be of an aggressive nature.
A complete cure for this type has not yet been invented; long-term remission can be obtained. Currently, life expectancy with this diagnosis is on average 6 years. Previously they had not reached three.
Main organs affected: spleen, bone marrow, peripheral blood, extranodal areas.
Based on primary signs, it can also be determined fairly quickly. Secondary are pain in the abdomen, joints, bones. There may also be bone deformities of the limbs and spine.
A blood test reveals the cellular composition of the blood
Diagnostics
A biopsy involves taking lymphoid tissue from the affected node for further analysis.
To make an accurate diagnosis, the doctor needs to conduct a comprehensive examination of the patient. Usually it involves a certain algorithm:
- Examination of the patient. The doctor listens to complaints, clarifies the medical history and palpates the affected areas.
- Laboratory diagnostics. To confirm suspicions of the development of large cell lymphoma, it is necessary to donate blood for biochemical and clinical tests. The latter allows you to determine the number of lymphocytes, as well as the erythrocyte sedimentation rate. Biochemical analysis determines the content of urea, glucose, total lipids and other parameters in the patient’s blood. Thanks to this procedure, it is possible to identify the presence of functional disorders in the body.
- Biopsy. The main stage of diagnosis. The procedure involves collecting lymphoid tissue from the affected node, which is necessary for subsequent histological examination. The result allows you to determine the presence of malignant cells in the body.
- Radiation diagnostics. We are talking about both the standard radiography procedure and more precise techniques: CT and MRI. With the help of radiation diagnostics, a specialist is able to identify the stage of development of the disease and determine the presence of malignant neoplasms.
- Additional research. Depending on the degree and nature of the pathology, the patient may be prescribed a molecular genetic or immunohistochemical study.
Based on the results of the procedures described above, the doctor makes a final diagnosis and develops an individual treatment plan for the patient.
Diagnostic measures
An unspecified tumor is not treated. It is important to differentiate the cells of the lesion and clarify the diagnosis. Oncologists prescribe a set of instrumental diagnostic studies:
- Clinical tests of urine and blood.
- Ultrasound diagnostics of abdominal organs and lymph nodes of the neck, collarbone, groin and other areas.
- To clarify the condition of the mediastinum area, respiratory organs and bone tissue, radiography is performed.
- Determination of tumor markers, in particular, Beta-2-microglobulin titers and protein compounds.
- CSF and bone marrow punctures.
- Magnetic resonance and computed tomography.
- Histological examination of tumor tissue provides the final diagnosis.
Features of treatment
The most common treatments for diffuse large B-cell lymphoma are radiation and chemotherapy. As a rule, they are used together, which allows you to get the best results.
Chemotherapy
Dacarbazine is administered intravenously, and in some localizations of the tumor process intra-arterially
The technique is based on the introduction into the body of potent substances that have a destructive effect on cancer cells. This treatment gives good results when used in the first stages of the disease. The most commonly used drugs during chemotherapy are:
- Vinblastine;
- Bleomycin;
- Doxorubicin;
- Dacarbazine.
In most cases, treatment is carried out in two stages. The first is the basis of therapy, and the second consolidates the effect obtained.
If the patient has an unfavorable prognosis, the treatment approach changes. In this case, the following drugs are prescribed:
- Cyclophosphamide;
- Onkovin;
- Doxorubicin.
In advanced stages of the disease, intensive therapy is carried out, the goal of which is to achieve absolute remission.
Chemotherapy is also used to treat generalized stages of primary mediastinal B-cell lymphoma.
Radiation therapy
Involves the use of X-rays to destroy pathologically altered cells. Unlike chemotherapy, which is used to treat all stages of the disease, this technique is used for patients with the first and second stages of lymphoma.
A single radiation dose does not exceed 40 gray. In this case, the rays are directed only to the affected area. Other tissues should not be affected.
The main goal of this treatment is to damage the structure of malignant cells and inhibit their growth. The radiation dose is determined by the stage of the disease. Possible risk factors are also taken into account. Thus, with significant tumor metastasis, radiation therapy may be contraindicated, since such an effect on the affected areas will only lead to the spread of metastases.
Drug therapy
The total dose of the drug should not exceed 1000 mg/m2 of body surface, administered intravenously, slowly, over 3-5 minutes
The following groups of drugs are used as accompanying therapy in the treatment of large cell lymphoma:
- antibiotics if the disease is bacterial in nature;
- antitumor agents to inhibit the growth of tumors;
- immunomodulators – to increase the body’s protective functions;
- antiviral drugs if the inflammatory process was triggered by the activity of a virus.
The use of additional funds should be carried out under the close supervision of a specialist. If any changes in well-being occur, the patient should immediately contact the attending physician in order to adjust the treatment plan.
The most popular drugs used as part of adjuvant therapy are cytostatics and drugs from the group of monoclonal antibodies:
- Vinblastine;
- Methotrexate;
- Epirubicin;
- Asparaginase;
- Rituximab;
- Etoposide.