Lymphosarcoma is a pathological neoplasm arising from the reticular connective tissue, from which all the blood-forming organs and lymphoid clusters in the mucous membranes are composed. Therefore in modern sources this disease has received the name reticulosarcoma. Cells of this tumor outwardly resemble absolutely healthy reticular cells. However, in histochemical analysis, in the tissues of lymphosarcoma, in large quantities are argyrophilic fibers absorbing silver.
All types of lymphosarcoma are characterized by rapid progression and metastasis in the lymphatic and circulatory systems. One of the most common lymphosarcomas is reticulosarcomatosis, which in the clinical picture almost resembles acute leukemia. For the treatment of lymphosarcomas, chemotherapy and radiation are used.
To date, the final causes of the emergence of lymphosarcoma are practically not found. To them it is possible to attribute some factors contributing to the development of this pathological process.
First, these are various diseases with congenital and acquired immunodeficiency signs. The first such pathologies include Wiskott-Aldrich syndrome, Chediak-Higashi, Klinefelter , ataxia-telantiectasia and variable immunodeficiency state. And to the acquired - HIV infection, hapogammaglobulinemia, iatrogenic immunosuppression.
Thirdly, when exposed to the body of various chemicals and drugs (dioxin, phenytoin and herbicide).
Also, the formation of lymphosarcoma can be affected by chemotherapy, radiation and ionizing radiation. Among the infectious agents in the progression of the malignant tumor, Epstein-Barr viruses, human T-cell leukemia and Helicobacter are isolated.
Many patients with lymphosarcoma often display anomalies at the cytogenetic level. It was found that with changes in chromosomes, the genes control the formation of light and heavy chains of immunoglobulins, a shift occurs to the genes responsible for differentiation and proliferation. It has already been proved that such oncological genes can not control the work of healthy B lymphocytes.
According to the incidence of lymphosarcomas, hemolytalosis of malignant etiology is 15%, which is only slightly inferior to lymphogranulomatosis. Basically, this disease has two kinds of origin: B-cell and T-cell.
Among the histological forms of lymphosarcoma are isolated: nodular, immunoblastic, prolymphocytic, lymphoplasmocytic, lymphocytic and lymphoblastic.
This malignant formation does not distinguish certain age categories. However, with lymphosarcoma there is a greater susceptibility of men than women. Clinical manifestations of the disease with its first signs largely depend on the primary localization of the tumor process. First of all, the defeat of lymphosarcoma depends on the B-cell or T-cell type of the disease.
Lymphosarcoma of the mediastinum, which is a B-cell primary form, usually affects women at a young age. This tumor for a long time does not leave its affected hearth and is located in the anterosurface of the mediastinum. In this case, the symptoms of intoxication are not typical for this type of lymphosarcoma, even with significant dimensions of the pathological focus. But sometimes there may be a concomitant compression syndrome, which forms in the hollow upper vein and is the only sign on which it is possible to establish a lymphosarcoma. Therefore, the main role in the diagnosis of the disease is the accidental detection of a tumor on the radiographic examination of the lungs.
To such early signs of lymphosarcomas, as concomitant viral and inflammatory diseases, include general malaise, fever and cough. With the progression of the malignant process, the person acquires swelling, a characteristic shortness of breath appears, which increases with physical exertion, and also the veins on the chest. Subsequently, the tumor spreads into the pericardium, the lungs, while damaging nearby tissues.
Lymphosarcoma of the mediastinum can give metastases to the adrenal glands, pleura, kidneys, brain and other organs located in the retroperitoneal space. Much less often the disease spreads to the lymph nodes, bone marrow and spleen. Therefore, to confirm the diagnosis, an important analysis is taking a biopsy. A common clinical symptom of this type of lymphosarcoma is sclerosis, to which its nodular type can be attributed.
One of the most common types of lymphosarcoma is the B-cell primary tumor with involvement of peripheral lymph nodes. The clinical picture is characterized by multiple manifestations, and this makes it possible to determine its subsequent development. In general, the symptomatology is manifested by all signs of intoxication and a predominant increase in supraclavicular lymph nodes. They form conglomerates, becoming rather dense at palpation. As a result of the fact that the lymphosarcoma can germinate into various tissues nearby, then there is a kind of tumor that has conglomerates with a woody and immobile density without definable boundaries.
Further tumor germination can affect the spleen and other extranodal organs (CNS and bone marrow). In some cases, lymphosarcoma of this species can block the lymphatic flow and compress the venous trunks. And with the possible germination of the pathological process in the kidneys and squeezing the ureter, hydronephrosis develops. This compression of the nerve endings and the germination of the tumor causes severe pain in patients. All these symptoms, squeezing and germination, indicate a far-advanced disease progression that determines bad predictions in the future.
Symptomatic lesions of mesenteric lymph nodes with B-cell lymphosarcoma of the primary form is characterized by the appearance of rapid depletion of the body due to profuse diarrhea. To this sign of the disease is added more ascites, with a characteristic accumulation of fluid in the abdominal cavity. Then at palpation it is possible to determine a significant increase in lymph nodes. And the concomitant symptom of the disease is the enlarged spleen.
Later lymphosarcoma gives metastases to the intestines, which is characterized by its obstruction, and this is the signal for urgent hospitalization of patients according to the indications. If the pathological process is localized outside the intestine, then this leads to an increased volume of the abdomen. This clinical picture is the consequence of severe stages of the disease. Thus, the general symptomatology in the early stages of pathological education does not have significant manifestations, therefore patients are not in a hurry to consult a doctor, which subsequently causes the tumor to be determined already in the late stages of the disease.
When lymph nodes are affected with lymphosarcoma, there is a distinctive feature that says that these oncological changes have the highest percentage of cure achieved by the action of polychemotherapy and radiation exposure. After acquiring lymph nodes of normal size, diarrhea passes, and this normalizes the patient's body weight.
When the spleen is affected by the primary B-cell lymphosarcoma, this organ first increases. Then there is a heaviness and pain in the left hypochondrium with periodic increases in temperature. For this lymphosarcoma is characterized by a syndrome of hypersplenism. For a long time, metastases can not penetrate the bone marrow. Therefore, to establish an accurate diagnosis, a splenectomy is prescribed. And for her, not only therapeutic functions, but diagnostic ones are also characteristic. In addition, spleen removal is carried out at increased sizes with absent tendencies to cure.
The clinical picture of brain lymphosarcoma has no characteristic differences from other types of tumors. Symptomatology consists of focal and neurologic general cerebral manifestations. This is characterized by central paresis and paralysis, as well as increased intracranial pressure. In most cases, concomitant disease in the emergence of such a lymphosarcoma, is HIV infection, as a result of which this pathology is manifested.
The spread of T-cell tumors in the body is much less than that of B-cell forms. In general, the clinical picture of such groups of malignant pathology does not substantially differ. However, T-cell lymphosarcoma in its progression takes a leading place and develops rapidly, and this worsens the prediction of the disease.
In very rare cases, lymphosarcoma penetrates the skin, so this area of damage has not been fully explored. As a rule, the tumor is localized in the lower layers of the dermis, and then spreads throughout the cellulose. It has dense formations, it is motionless and does not change its coloring at all. When taking skin patches on a biopsy, deep and long-lasting ulcers remain.
The purpose of treatment for lymphosarcoma is reduced to individualization and depends on the structure of the tumor, its spread into tissues and organs, the possibilities of healing, as well as the available accompanying pathologies and the patient's age.
In very rare cases, surgical intervention is used when lymphosarcoma affects extranodal organs, for example, the gastrointestinal tract. But this treatment is only one of the components of general therapy.
The basis of the entire therapeutic process for lymphosarcoma is polychemotherapy, which makes it possible to achieve not only clinical remission, but also hematological, and also to fix it in an antirecurrent course.
The use of local radiotherapy has justified itself in conjunction with drug therapy or with palliative irradiation of pathological formations. Only in the first stage of lymphosarcoma radiation therapy is shown, as an independent method of treatment, and then at low degrees of malignancy, as well as when spreading the process to the bone. This kind of radiation irradiation or surgical excision of the tumor maintains an absolute remission in many patients up to ten years.
Lymphosarcoma in the generalized process proceeds slowly enough.
Monochemotherapy, which includes Cyclophosphamide, Pafenzil, Chlorbutin, is prescribed for elderly patients with a low degree of malignancy of lymphosarcoma, with concomitant other serious diseases. The purpose of splenectomy is used in primary lesions of the spleen, regardless of the morphological type of the disease. Then a course of polychemotherapy is carried out. With lesions of the skin, chemotherapy with Prospidin and Spirobromin is prescribed.
For the third and fourth stages of lymphosarcomas of an aggressive form, chemotherapy cycles are carried out with further monitoring treatment. And localized radiotherapy is applied to more affected tumor sites, which is effective in the lymphosarcomas of the pharyngeal ring in the primary form. In order to exclude the generalization of a malignant tumor, irradiation with polychemotherapy is combined.
Also, with a low degree of malignancy of the pathological process for the generalized stages of lymphosarcoma, programs are appointed: BATSOP, TSOPP, TSOP, etc.
The results of cytostatic treatment of lymphosarcoma largely depend on the timely use of hematological drugs, immunocorrecting agents and antibacterial therapy.
In some cases with positive hematopoiesis, polychemotherapy can be performed in outpatient settings, which greatly facilitates the treatment of patients. This is due to the psychological effects of hematological and oncological hospitals. In addition, after the achieved remission, for two years with interruptions of three months, anti-relapse treatment is conducted before the onset of persistent positive dynamics in the clinical-hematological form.
The prognosis of lymphosarcoma largely depends on the stage with which the treatment was started. After a timely and early diagnosis of the disease, it is possible to increase in half the cases the duration of remissions. During the therapy of lymphosarcomas with minor lesions, success is possible in 80-100%.
But the prognosis for disseminated form and generalized lymphosarcoma is unfavorable. And often the treatment of this malignant disease is ineffective, with a possible short-term effect.