Lymphoma is the combined name of several oncological diseases, which tend to affect the lymphatic tissue with an increase in lymph nodes and the defeat of many somatic organs, which are characterized by uncontrolled accumulation of pathological lymphocytes. Lymphoma, like all solid tumors, is manifested by the presence of the primary form of the tumor focus. However, lymphoma can not only give metastases, but also spread to all systems of the body with the simultaneous development of a condition that resembles lymphatic leukemia . In addition, there is lymphoma without enlarging the lymph nodes. It can primarily develop in various organs (lungs, brain, stomach, intestines). This lymphoma refers to extranodal forms.
Lymphoma is of two kinds. This is a large group of non-Hodgkin's lymphomas and Hodgkin's lymphoma. In microscopic studies, specific Berezovsky-Sternberg cells found that indicate the diagnosis of Hodgkin's lymphoma , and if they are absent, the diseases are referred to as non-Hodgkin's lymphomas.
Lymphoma of the cause
To date, it has not been possible to establish the specific causes of the appearance of different forms of lymphomas. To date, many toxic substances have been studied with respect to involvement in the formation of pathological diseases. However, there is no convincing evidence that these substances can provoke the onset of lymphoma.
There are assumptions of the relationship between some risk factors and the probability of the development of these pathologies in a certain category of people. This group includes people who have lain or are sick with close relatives of lymphomas; suffering from autoimmune diseases; those who have undergone stem cell or kidney transplant surgery; working with carcinogenic substances; infected with Epstein-Barr viruses, AIDS, hepatitis C, T-lymphotropic type and herpes; having in the presence of bacteria Helicobacter pylori. Thus, the infection of the body with various bacteria and viruses, the nature of professional activity can cause the appearance of lymphoma in humans.
Also, the risk of developing the disease in people with weakened immunity is greatly increased, which is due to the inherent or acquired defect.
All clinical manifestations of the disease will depend on its type and localization. Hodgkin's lymphoma includes five types of malignant pathologies and almost thirty subtypes of non-Hodgkin's lymphomas.
Lymphoma of the first type is distinguished by a variety of clinical symptoms, which are characterized by lesions of different types of lymph nodes and organs. All the symptoms of the disease are divided into common manifestations of the disease and local. Practically every third patient with this pathology reveals a general picture of lymphoma, which is a rise in temperature, profuse night sweats, weakness in the whole body, pain in the joints and bones, fatigue, weight loss, headaches and itching on the skin . One of the earliest signs of the disease is the change in the temperature regime in the body.
Lymphoma at the very beginning of progression is characterized by the fact that the temperature rests on the low-grade figures, and the characteristic increase begins to occur precisely in the evening. As a result of how lymphoma continues to gain progression, the temperature reaches 39-40 degrees, and at night the patients experience a chill that turns into profuse sweating.
One of the symptoms of lymphoma is the general weakness of the patient, which reduces the ability to work and can appear even before the diagnosis of the disease.
Lymphoma of the skin manifests itself as a characteristic symptom in the form of skin itch with varying degrees, which can appear long before lymphatic damage to organs and nodes. Therefore, it can for a long time remain the only confirmation of the disease. Itching is a generalized spread across the patient's body with possible localization in some parts, such as the anterior surface of the thoracic region, on the head, limbs, including the feet and palms.
When examining patients, it is possible to diagnose various lesions of lymph nodes with diverse localization. In almost 90% of the pathological foci are above the diaphragm, and the remaining 10% are observed in the lower parts of the lymphocytes.
Typically, lymphoma in 70%, which refers to Hodgkin's lymphoma, manifests with an increase in cervical lymph nodes, which are characterized by an elastic consistency, not soldered to each other and to nearby tissues. When palpation, they are absolutely painless, and the skin of the tumor conglomerate is not changed, without redness and infiltration. Also in 25% of the lymphoma can affect the supraclavicular lymph nodes, which are generally never large. About 13% are affected by lymph nodes in the armpit areas, which, with their anatomical structure, contribute to the rapid spread of the pathological process to the mediastinal lymphatic system, as well as to the lymph nodes located under the breast muscle with the transition to the mammary gland.
Lymphoma of the mediastinum is manifested by the involvement of lymph nodes in 20%. Clinically, this manifests itself as a pathological change not only in the lymph nodes, but also in the processes of compression and their germination into other organs and tissues. At the time of onset, there is a slight dry cough, which is accompanied by pain in the thoracic region. These pains can be of different localization and appear constant and blunt, which can intensify during a deep inspiration or cough. Sometimes patients complain about pain in the area of the heart, stabbing character, and a bursting feeling in the thoracic area. This is due to the increase in lymph nodes, which begin to press on the nerve endings and the heart or their germination. In this case, mediastinal lymphoma can spread into pericardium, lungs and bronchi. After this, patients experience a slight dyspnea, which develops during physical exertion or ordinary walking. And with the further progression of the disease, i.e. growth of lymph nodes, dyspnea is much worse. If the lymph nodes of the mediastinum increase to a considerable extent, then the hollow vein syndrome in the upper part can develop. Although there are cases of asymptomatic course of this pathology, which is accidentally diagnosed with a planned X-ray examination of the chest organs.
Retroperitoneal lesions of lymph nodes are extremely rare and amount to about 8% of cases. Most often, these lesions cause the development of gastric lymphoma. The onset of the disease is characterized by an asymptomatic flow pattern and only with a slight increase in lymph nodes in this area, pain and numbness of the lumbar region, flatulence and constipation appear. When you drink alcohol, even in minor amounts of pain in this area is somewhat strengthened.
Lymphoma, which affects lymph nodes in the inguinal and iliac regions, occurs in 3% of cases, but differs in poor prognosis and malignant course. Changes that occur in these lymph nodes cause cramping or persistent pain in the abdomen. Frequent signs of the defeat of these lymph nodes is a disturbed lymph drainage, heaviness in the limbs, decreased skin sensitivity inside and in front of the hips, an edema of the feet.
Sometimes doctors diagnose spleen lymphoma, which is considered a rare pathology with relatively benign course and a high life expectancy of patients, when compared with other forms. Lymphoma can involve the spleen in the tumor process in 85% of cases. If the pathology of this organ is affected, its increase is noted as the only sign of the disease, which is determined by ultrasound or by radionuclide research. However, its normal dimensions are not that changes are absent or vice versa.
Lymphoma of the lungs in the primary form is very rare, therefore some authors question its occurrence. However, in 30% of cases, the lungs are affected by an anomaly and are attached to the tumor process. Malignant cells can enter the lung tissue through lymph or blood from pathological foci as a result of the formation of lymphadenitis with a direct transition of the process from the lymph nodes of the mediastinal divisions to the lung tissue. Clinically, this is manifested by coughing, shortness of breath, chest pains and in some cases hemoptysis. If lymphoma captures pulmonary tissue in limited quantities, then cough is present insignificant, and all other signs are completely absent. The defeat of the pleura, which is accompanied by an effusion in the pleural cavity, is characterized by specific changes in the lungs.
In about 30% of cases, lymphoma affects the bones. There is lymphoma with primary and secondary damage to bones as a result of germination from pathological foci of nearby structures or through blood. Most often, lymphoma involves the spine, then the ribs, sternum and pelvic bones in the process of lesion. It is very rare to observe pathological changes in tubular bones and skull bones. But if this happens, the patients complain of characteristic pain. When the tumor is localized in the vertebrae, the pain manifests itself in an irradiating character, which is amplified by pressing on the vertebrae. With lesions of the vertebrae of the lower thoracic and upper lumbar parts, numbness in the legs and twitching appear. With further progression of the process, paralysis and paresis of the lower limbs are revealed, as well as the functions of the organs that are located in the pelvic region.
Lymphoma in 10% affects the liver. A pathological lesion of the organ can be either single or multiple. Symptomatic of such changes is manifested in the form of nausea, heartburn, heaviness in the right side of the hypochondrium and an unpleasant aftertaste in the mouth. In such patients, signs of jaundice of different origin are detected, and this aggravates the prediction of the disease.
Lymphoma of the brain is not marked by specific changes, and such lesions are determined in 4% of cases.
In addition, there are lesions in the tumor of other tissues and organs. It can be lymphoma of the thyroid gland, breast, heart muscle, nervous system.
The lymphoma can proceed either aggressively or indolently, but sometimes a highly aggressive course with a rapid spread of the malignant tumor is noted. Non-Hodgkin's lymphomas are characterized by aggressive course with high malignancy. Lymphoma with a low malignancy is an indolent flow. The prognosis of these lymphomas is common. Lymphoma with aggressive course has more opportunities for recovery, but indolent forms belong to incurable pathologies. In addition, they are well treated with polychemotherapy, radiation and surgical intervention, but, nevertheless, they have a pronounced predisposition to relapse, as a result of which death often occurs. At any stage, this lymphoma can go to a diffuse B-large cell with subsequent damage to the bone marrow. Such a transition is called Richter syndrome, which indicates a survival rate of up to twelve months.
Malignant lymphoma, as a rule, first affects the tissue of the lymph, and then - the bone marrow. This is what distinguishes her from leukemia.
Lymphoma, which belongs to the non-Hodgkin group, occurs in peripheral and visceral lymph nodes, thymus, lymphoid nasopharyngeal and gastrointestinal tract. Much less often it affects the spleen, salivary glands, orbit and other organs.
Lymphoma is also nodal and extranodal. It depends on where the tumor first localized. But since malignant cells spread very quickly over the body, malignant lymphoma is characterized by a generalized arrangement.
Malignant lymphoma is characterized by an increase in one lymph node or several; the presence of extranodal damage and the general onset of the pathological process in the form of weight loss, weakness and fever.
Lymphoma of stage
With the help of stages it is possible to determine the possibility of penetration and spread of malignant neoplasm in the human body. The information obtained helps to make a correct decision in the appointment of an appropriate therapeutic treatment program.
Based on the conventional classification of Ann-Arbor, four stages of the malignant process are distinguished.
The first two stages of lymphoma are conventionally considered local or local, and the third and fourth are common. To the numbers (I, II, III, IV), if the main three symptoms (night sweats, fever and weight loss) are present, add the letter B, and in the absence of the letter A.
At the first stage of lymphoma, one area of the lymph nodes is involved in the tumor process;
In the II stage of lymphoma, several regions of the lymph nodes are affected, which are localized only on one side of the diaphragm;
At the third stage of lymphoma, lymph nodes are localized on both sides of the diaphragm;
At the IV stage, lymphoma spreads to various somatic organs and tissues. With a massive lesion of the lymph nodes to the stage, the symbol X is added.
This lymphoma refers to aggressive forms of the disease, in which the structure of the lymph node is broken and cancer cells are located in all areas.
B-cell lymphoma is one of the most common types of non-Hodgkin's lymphomas with high malignancy, a variety of morphological characteristics, clinical symptoms and their sensitivity to treatment methods. This can be explained by the fact that B-cell lymphoma can develop both primarily and transform from non-Hodgkin's non-Hodgkin's lymphomas, for example, from follicular lymphoma, MALT-lymphoma. Pathological cells are characterized by phenotypic signs of centroblasts or immunoblasts as a result of expression of B-cell antigens. In 30% of cases, a cytogenetic abnormality is observed, which is called translocation (14, 8). With B-cell lymphoma, the gene is reanimated (in 40%) or its mutation (in 75%).
The first two stages of the disease can be diagnosed in one third of patients, and the remaining cases are disseminated and the pathological process involves extralymphatic zones.
B-cell lymphoma is formed from immature precursor cells of B-lymphocytes. This disease consists of several forms, including Burkitt's lymphoma, chronic etiologic lymphocytic leukemia, diffuse large-cell B-cell lymphoma, immunoblastic large-cell lymphoma, follicular lymphoma, B-lymphoblastic precursor of lymphoma.
B-cell lymphomas develop at a rapid rate. Depending on the location of the localization, there are different kinds of symptoms. In the first place are enlarged lymph nodes, which do not cause painful sensations. They can be formed on the neck, hands, in the armpits, in the head region or in several zones simultaneously. The disease also develops in the cavities, where it is very difficult to determine the increase in lymph nodes. Then the disease sprouts into the spleen, liver, bones and brain.
Simultaneously there is a fever, weakness, sweating at night, weight loss, fatigue. Symptomatic with B-cell lymphoma progresses for two to three weeks. With a typical clinical picture, a specialist should be consulted and a diagnostic examination performed to exclude or confirm the diagnosis.
For the treatment of lymphoma, traditional methods of treatment of oncological diseases are used, which include radiation irradiation and polychemotherapy, as well as their combinations.
When choosing methods of treatment, the stages of lymphoma and risk factors that contribute to the development of the pathological process are taken into account. The most important risk criteria include altered lesions of the lymphatic area (more than three); at Stage B - the erythrocyte sedimentation rate is 30 mm / h, at stage A - 50 mm / h; lesions of extranodular nature; extensive defeat of the mediastinum; at МТИ 0,33; with massive splenomegaly with diffuse infiltration; lymph nodes - more than five centimeters.
Treatment of patients with a diagnosis of lymphoma begins with the appointment of polychemotherapy drugs. And only patients with the first stage (A) receive radiation irradiation of the affected lesion in a total dose of 35 Gy. Virtually all patients with intermediate and favorable prognosis are assigned polychemotherapy ABVD in the form of a standard scheme, and in case of an unfavorable prognosis - VEASORR. The first scheme includes intravenous administration of such drugs as Dacarbazine, Bleomycin, Doxorubicin and Vinblastine. The second scheme includes: Bleomycin, Prednisolone, Etoposide, Cyclophosphamide, Vincristine, Doxorubicin and Procarbazine.
The group with a favorable prognosis includes the first two stages of lymphoma without risk factors. Such patients begin to be treated with the appointment of two ABVD courses. After the completion of polychemotherapy, radiation irradiation is carried out after three weeks. The intermediate prognosis group includes the first two stages with at least one risk factor. For such patients, four courses of ABVD are first conducted, and after a few weeks, radiation irradiation of the initially affected areas is undertaken. The group with unfavorable prognosis includes the last two stages (III, IV) of the pathological process. Here treatment begins with VEASORR or ABVD with obligatory rest in two weeks. Then, radiotherapy is also given with irradiation of the affected skeleton.
In the treatment of patients with indolent and aggressive forms of the disease, the main tasks, such as maximum survival of patients and improvement of their quality of life, are attempted. Success in solving these problems depends on the type of lymphoma itself and the stage of its course. For a localized course of malignant pathology, an important point is eradication of the tumor, an increase in life expectancy, and a possible cure. In the generalized process, rational treatment is prescribed in the form of anticancer therapy and palliative treatment, which are associated with improving the quality of life and increasing its duration.
The terminal stage of lymphoma is characterized by a program of palliative therapy, for which it is important to improve the quality of life of patients. The basis of palliative therapy includes: psychological support, spiritual, social, symptomatic and religious.
Lymphoma of malignant form and aggressive nature of the course with the presence of favorable prognosis is cured in 35%. It is characterized by individual prediction when using a tumor scale or on the basis of MPI estimation. According to the MPI system, each adverse feature is one point. When summing prognostic scores determine the course of the disease, favorable or unfavorable. With the total number of points from zero to two, it is lymphoma with a favorable prognosis, from three to five - unfavorable, from two to three - indefinite. When a significant number of patients with an uncertain prognosis are allocated, a more complete tumor scale assessment is used, which includes parameters such as stage and general symptoms, tumor size, LDH and microglobulin levels.
Adverse symptoms on this scale are the last two (III, IV) stages, B-symptoms, lymphoma more than seven centimeters, 1.1-fold increased LDH level, and the microglobulin level is exceeded by 1.5 times. Patients whose prognostic total scores exceed the number of three refer to patients with poor prognosis, and less than three to the group of favorable prognosis. All these data are used in the appointment of individual treatment.
For the treatment of patients with aggressive forms of lymphomas of a favorable prognosis, chemotherapeutic treatment in the form of an ASOP or CHOP scheme is prescribed. It includes such drugs as Prednisolone, Doxorubicin, Vincristine (Onkovin), Cyclophosphamide. The main goal of polychemotherapy is to achieve absolute remission in the first stages of therapy, since this is related to the indicators of overall survival improvement. In partial forms of regression of a pathological neoplasm, polychemotherapy is always supplemented by radiation irradiation of affected regions.
The problem group of treatment includes elderly patients, who are characterized by the dependence of the effect of therapy on age. Up to forty years, complete remissions are 65%, and after sixty - about 37%. In addition, toxic mortality can be observed up to 30% of cases.
For the treatment of patients in old age, Rifaximab is added to chemotherapeutic drugs, which almost triples the average survival rate. And for patients under 61 years of age, consolidating teletherapy and PCT are used, using the R-CHOP scheme.
Recurrent treatment of aggressive lymphomas, which are characterized by a generalized course of the disease, depend on many factors. This concerns the histology of the tumor, previous therapy and sensitivity to it, the response to treatment, the age of the patient, the overall somatic status, the state of some systems, and the bone marrow. Typically, treatment of a relapse or progressive process should include previously not used drugs. But sometimes in the treatment of relapses that developed one year after the achieved absolute remission, using the initial treatment regimens, can give good results.
Lymphoma in the second stage of the disease, with large tumor sizes, with lesions in more than three regions, with B-cell form and with unfavorable values of MPI has a greater risk for the development of early relapses.
There is such a thing in medicine as the therapy of despair. This type of treatment is used for patients with primary refractory forms and with early relapses of malignant anomalies in the form of increased doses of polychemotherapy. Remission in the therapy of despair occurs less in 25% of cases, and they are quite short.
The appointment of high-dose therapy is considered an alternative in the treatment of severe patients. But it is appointed with a good obscheomatic status.
Lymphoma with aggressive course is amenable to treatment with high-dose therapy for the first relapses of tumor pathology.
At the heart of the prognostic factor of lymphoma of the indolent species with malignancy of a low degree lies the stage of the disease. Thus, the staging of pathology is determined after bone marrow biopsy of the bone marrow as a result of its strong involvement in the disease process.
To date, lymphoma in indolent phoma has no specific standards in treatment, since they are absolutely equally sensitive to known methods of treatment of cancer, and as a result of cure does not occur. The use of polychemotherapy leads to short-lived positive results, and then the disease begins to recur. The use of radiotherapy as an independent treatment is effective at the first or first (E) stage of lymphoma. For tumors of five centimeters, the total dose is up to 25 Gy on the pathological focus and this is considered sufficient. At the last three stages of the pathological disease, radiation polycondensation in 35 Gy is supplemented with polychemotherapeutic drugs. Sometimes in 15% of indole lymphoma can suddenly regress. Then they start treatment with standard schemes. Chlorbutin mono-chemotherapeutic agent with Prednisolone can be used. And also use polychemotherapy in the form of a CVP scheme, which includes Cyclophosphane, Vincristine, Prednisolone.
The final therapy is radiation exposure, prescribed according to the indications. In the form of maintenance therapy, Interferon is used.
Five-year survival is achieved using modern methods of polychemotherapy, as well as radiotherapy. For example, in patients with favorable prognosis, such results can be achieved in 95%; with an intermediate - in 75% and with an unfavorable forecast - in 60% of cases.