Prognosis for life in non-Hodgkin's lymphoma. Features of the clinical picture in non-Hodgkin's lymphoma Non-Hodgkin's lymphoma stage 4 prognosis

- Tumor diseases of the lymphatic system, represented by malignant B- and T-cell lymphomas. The primary focus can occur in the lymph nodes or other organs and further metastasize by the lymphogenous or hematogenous route. The clinic of lymphomas is characterized by lymphadenopathy, symptoms of damage to one or another organ, fever-intoxication syndrome. Diagnosis is based on clinical and radiological data, the results of a hemogram study, biopsy of lymph nodes and bone marrow. Antitumor treatment includes courses of polychemotherapy and radiation therapy.

ICD-10

C82 C85

General information

Non-Hodgkin's lymphomas (NHL, lymphosarcomas) are malignant lymphoproliferative tumors that differ in morphology, clinical signs and course, differing in their characteristics from Hodgkin's lymphoma (lymphogranulomatosis). Depending on the place of occurrence of the primary focus, hemoblastoses are divided into leukemias (tumor lesions of the bone marrow) and lymphomas (tumors of lymphoid tissue with primary extra-medullary localization). Based on the distinctive morphological features of lymphoma, in turn, are divided into Hodgkin's and non-Hodgkin's; among the latter in hematology include B- and T-cell lymphomas. Non-Hodgkin's lymphomas occur in all age groups, but more than half of cases of lymphosarcoma are diagnosed in people over 60 years of age. The average incidence among men is 2-7 cases, among women - 1-5 cases per 100,000 population. In recent years, there has been a trend towards a progressive increase in the incidence.

The reasons

The etiology of lymphosarcoma is not known for certain. Moreover, the causes of lymphomas of different histological types and locations vary significantly. Currently, it is more correct to talk about risk factors that increase the likelihood of developing lymphoma, which are currently well studied. The influence of some etiofactors is pronounced, the contribution of others to the etiology of lymphomas is very insignificant. These unfavorable conditions include:

infections. Human immunodeficiency virus (HIV), hepatitis C, T-lymphotropic virus type 1 has the greatest cytopathogenic effect on lymphoid cells. Epstein-Barr virus infection has been shown to be associated with the development of Burkitt's lymphoma. It is known that Helicobacter pylori infection associated with gastric ulcer can cause the development of lymphoma of the same localization.
Immunity defects. The risk of developing lymphomas increases with congenital and acquired immunodeficiencies (AIDS, Wiskott-Aldrich syndrome, Louis Bar, X-linked lymphoproliferative syndrome, etc.). Patients receiving immunosuppressive therapy for bone marrow or organ transplantation are 30 to 50 times more likely to develop NHL.
Accompanying illnesses. An increased risk of NHL incidence has been noted among patients with rheumatoid arthritis, lupus erythematosus, which can be explained both by immune disorders and the use of immunosuppressive drugs for the treatment of these conditions. Thyroid lymphoma usually develops against the background of autoimmune thyroiditis.
Toxic effect. There is a causal relationship between lymphosarcomas and previous exposure to chemical carcinogens (benzene, insecticides, herbicides), UV radiation, and radiation therapy for cancer. Direct cytopathic action is exerted by cytostatic drugs used for chemotherapy.

Pathogenesis

Pathological lymphogenesis is initiated by one or another oncogenic event that causes disruption of the normal cell cycle. Two mechanisms can be involved in this - activation of oncogenes or suppression of tumor suppressors (antioncogenes). A tumor clone in NHL in 90% of cases is formed from B-lymphocytes, extremely rarely from T-lymphocytes, NK-cells or undifferentiated cells. Different types of lymphomas are characterized by certain chromosomal translocations, which lead to suppression of apoptosis, loss of control over the proliferation and differentiation of lymphocytes at any stage. This is accompanied by the appearance of a clone of blast cells in the lymphatic organs. Lymph nodes (peripheral, mediastinal, mesenteric, etc.) increase in size and can disrupt the function of nearby organs. With infiltration of the bone marrow, cytopenia develops. Growth and metastasis of the tumor mass is accompanied by cachexia.

Classification

Lymphosarcomas that primarily develop in the lymph nodes are called nodal, in other organs (palatine and pharyngeal tonsils, salivary glands, stomach, spleen, intestines, brain, lungs, skin, thyroid gland, etc.) - extranodal. According to the structure of the tumor tissue, NHL are divided into follicular (nodular) and diffuse. According to the rate of progression, lymphomas are classified into indolent (with a slow, relatively favorable course), aggressive and highly aggressive (with rapid development and generalization). If untreated, patients with indolent lymphomas live an average of 7-10 years, with aggressive ones - from several months to 1.5-2 years.

The modern classification includes over 30 different types of lymphosarcomas. Most tumors (85%) originate from B-lymphocytes (B-cell lymphomas), the rest from T-lymphocytes (T-cell lymphomas). Within these groups, there are different subtypes of non-Hodgkin's lymphomas. The group of B-cell tumors includes:

diffuse large B-cell lymphoma- the most common histological type of lymphosarcoma (31%). It is characterized by aggressive growth, despite this, in almost half of the cases it can be completely cured.
follicular lymphoma- its frequency is 22% of the number of NHL. The course is indolent, but transformation into aggressive diffuse lymphoma is possible. The prognosis of 5-year survival is 60-70%.
small cell lymphocytic lymphoma and chronic lymphocytic leukemia- close types of NHL, which account for 7% of their number. The course is slow, but poorly amenable to therapy. The prognosis is variable: in some cases, lymphosarcoma develops within 10 years, in others, at a certain stage it turns into a rapidly growing lymphoma.
mantle cell lymphoma- in the structure of the NHL is 6%. Only 20% of patients overcome the five-year survival limit.
Marginal zone B-cell lymphomas- are divided into extranodal (can develop in the stomach, thyroid, salivary, mammary glands), nodal (develop in the lymph nodes), splenic (with localization in the spleen). Differ in slow local growth; at early stages well give in to treatment.
B-cell mediastinal lymphoma- is rare (in 2% of cases), but unlike other types, it affects mainly young women 30-40 years old. In connection with the rapid growth causes compression of the mediastinal organs; cured in 50% of cases.
Waldenström's macroglobulinemia(lymphoplasmacytic lymphoma) - is diagnosed in 1% of patients with NHL. It is characterized by hyperproduction of IgM by tumor cells, which leads to an increase in blood viscosity, vascular thrombosis, and capillary rupture. It can have both relatively benign (with a survival rate of up to 20 years) and transient development (with the death of the patient within 1-2 years).
hairy cell leukemia is a very rare type of lymphoma that occurs in older people. The course of the tumor is slow, not always requiring treatment.
Burkitt's lymphoma- it accounts for about 2% of the NHL. In 90% of cases, the tumor affects young men under 30 years of age. The growth of Burkitt's lymphoma is aggressive; intensive chemotherapy allows to achieve a cure of half of the patients.
lymphoma of the central nervous system The primary CNS lesion may involve the brain or spinal cord. More commonly associated with HIV infection. The five-year survival rate is 30%.

Non-Hodgkin's lymphomas of T-cell origin are represented by:

T-lymphoblastic lymphoma or progenitor leukemia- occurs with a frequency of 2%. They differ in the number of blast cells in the bone marrow:<25% опухолевых клеток патология расценивается как лимфома, при >25% - like leukemia. It is diagnosed mainly in young people, the average age of the diseased is 25 years. The worst prognosis is T-lymphoblastic leukemia, the cure rate for which does not exceed 20%.
peripheral T-cell lymphomas including cutaneous lymphoma (Cesari syndrome, mycosis fungoides), angioimmunoblastic lymphoma, extranodal natural killer lymphoma, lymphoma with enteropathy, subcutaneous panniculitis-like lymphoma, anaplastic large cell lymphoma. The course of most T-cell lymphomas is rapid, and the outcome is poor.

Symptoms

Variants of clinical manifestations of NHL vary greatly depending on the localization of the primary focus, the prevalence of the tumor process, the histological type of tumor, etc. All manifestations of lymphosarcoma fit into three syndromes: lymphadenopathy, fever and intoxication, extranodal lesions. In most cases, the first sign of NHL is an increase in peripheral lymph nodes. At first they remain elastic and mobile, later they merge into vast conglomerates. At the same time, the lymph nodes of one or many areas can be affected. With the formation of fistulous passages, it is necessary to exclude actinomycosis and tuberculosis.

Such non-specific symptoms of lymphosarcomas, such as fever without obvious causes, night sweats, weight loss, asthenia, in most cases indicate a generalized nature of the disease. Among the extranodal lesions, non-Hodgkin's lymphomas of the Pirogov-Waldeyer ring, gastrointestinal tract, brain dominate, less often the mammary gland, bones, lung parenchyma, and other organs are affected. Nasopharyngeal lymphoma on endoscopic examination has the appearance of a pale pink tumor with tuberous contours. Often, the maxillary and ethmoid sinuses, orbit sprout, causing difficulty in nasal breathing, rhinophony, hearing loss, exophthalmos.

Primary testicular lymphosarcoma may have a smooth or bumpy surface, elastic or stony density. In some cases, swelling of the scrotum develops, ulceration of the skin over the tumor, an increase in the inguinal-iliac lymph nodes. Testicular lymphomas are predisposed to early dissemination with lesions of the second testicle, central nervous system, etc. Breast lymphoma on palpation is defined as a clear tumor node or diffuse breast compaction; nipple retraction is uncharacteristic. When the stomach is affected, the clinical picture resembles gastric cancer, accompanied by pain, nausea, loss of appetite, and weight loss. Abdominal lymphosarcomas can manifest themselves as partial or complete intestinal obstruction, peritonitis, malabsorption syndrome, abdominal pain, ascites. Skin lymphoma is manifested by itching, nodules and reddish-purple induration. The primary lesion of the central nervous system is more typical for patients with AIDS - the course of lymphoma of this localization is accompanied by focal or meningeal symptoms.

Complications

The presence of a significant tumor mass can cause compression of organs with the development of life-threatening conditions. With damage to the mediastinal lymph nodes, compression of the esophagus and trachea develops, SVC compression syndrome. Enlarged intra-abdominal and retroperitoneal lymph nodes can cause intestinal obstruction, lymphostasis in the lower half of the body, obstructive jaundice, and compression of the ureter. Sprouting of the walls of the stomach or intestines is dangerous by the occurrence of bleeding (in case of vascular erosion) or peritonitis (when the contents enter the abdominal cavity). Immunosuppression makes patients susceptible to life-threatening infectious diseases. High-grade lymphomas are characterized by early lymphogenous and hematogenous metastasis to the brain and spinal cord, liver, and bones.

Diagnostics

The issues of diagnosis of non-Hodgkin's lymphomas are within the competence of oncohematologists. Clinical criteria for lymphosarcoma are an increase in one or more groups of lymph nodes, intoxication, extranodal lesions. To confirm the proposed diagnosis, it is necessary to conduct a morphological verification of the tumor and instrumental diagnostics:

Study of the tumor cell substrate. Diagnostic operations are performed: puncture or excisional biopsy of lymph nodes, laparoscopy, thoracoscopy, aspiration puncture of the bone marrow, followed by immunohistochemical, cytological, cytogenetic and other studies of diagnostic material. In addition to diagnosis, establishing the structure of NHL is important for choosing treatment tactics and determining prognosis.
Imaging methods. An increase in mediastinal and intra-abdominal lymph nodes is detected using ultrasound of the mediastinum, radiography and CT of the chest, abdominal cavity. The examination algorithm according to indications includes ultrasound of the lymph nodes, liver, spleen, mammary glands, thyroid gland, scrotum organs, gastroscopy. For the purpose of tumor staging, an MRI of the internal organs is performed; lymphoscintigraphy and bone scintigraphy are informative in detecting metastases.
Laboratory diagnostics. It is aimed at assessing risk factors and the function of internal organs in lymphomas of various localizations. In the risk group, HIV antigen, anti-HCV is determined. Changes in peripheral blood (lymphocytosis) are characteristic of leukemia. In all cases, a biochemical complex is examined, including liver enzymes, LDH, uric acid, creatinine, and other indicators. B2-microglobulin can serve as a peculiar oncomarker of NHL.

Chemotherapy. Most often, treatment of lymphomas begins with a course of polychemotherapy. This method can be independent or combined with radiation therapy. Combined chemoradiotherapy allows to achieve longer remissions. Treatment continues until complete remission is achieved, after which another 2-3 consolidating courses are necessary. It is possible to include hormone therapy in cycles of treatment.
Surgical interventions. It is usually used for isolated lesions of any organ, more often - the gastrointestinal tract. If possible, operations are radical in nature - extended and combined resections are performed. In advanced cases, with the threat of perforation of hollow organs, bleeding, intestinal obstruction, cytoreductive interventions can be performed. Surgical treatment is necessarily supplemented with chemotherapy.
Radiation therapy. As a monotherapy for lymphomas, it is used only for localized forms and a low degree of malignancy of the tumor. In addition, radiation can also be used as a palliative method when other treatment options are not possible.
additional treatment regimens. Of the alternative methods, immunochemotherapy with the use of interferon, monoclonal antibodies has proven itself well. In order to consolidate remission, transplantation of autologous or allogeneic bone marrow and the introduction of peripheral stem cells are used.

Forecast and prevention

The prognosis for non-Hodgkin's lymphomas is different, depending mainly on the histological type of the tumor and the stage of detection. With locally advanced forms, long-term survival averages 50-60%, with generalized forms - only 10-15%. Unfavorable prognostic factors are age over 60 years, stages III-IV of the oncological process, involvement of the bone marrow, and the presence of several extranodal foci. At the same time, modern PCT protocols in many cases allow achieving long-term remission. Prevention of lymphomas correlates with known causes: it is recommended to avoid infection with cytopathogenic viruses, toxic effects, excessive insolation. In the presence of risk factors, it is necessary to undergo regular examination.

Non-Hodgkin's lymphoma- a whole group of more than 30 related diseases that do not have the characteristics of Hodgkin's disease. Lymphoma is a type of cancer that affects the lymphatic system, which consists of lymph nodes (small closed collections of lymphocytes) united by a system of small vessels.

The standardized incidence rates of lympho- and reticulosarcomas range from 2-6.9 in men and 0.9-5 in women.

Non-Hodgkin's lymphoma men get sick much more often than women, their age at the time of diagnosis varies widely.

The occurrence of non-Hodgkin's lymphoma

Etiology The origin of non-Hodgkin's lymphoma is unknown. It is believed that the cause is the penetration of a viral infection into the human body or due to suppression of the immune system, especially after organ transplants. Epstein-Barr virus likely causes Burkett's lymphoma, a type of non-Hodgkin's lymphoma.

The course of non-Hodgkin's lymphoma

Variants of non-Hodgkin's lymphomas (lymphosarcomas) are established in accordance with the WHO morphological classification, which are correlated with the degree of malignancy presented in the International Working Formulation of Non-Hodgkin's Lymphomas for Clinical Use.

Low-grade non-Hodgkin's lymphomas:

lymphocytic, diffuse type;
prolymphocytic, nodular type;
lymphoplasmacytic.

Non-Hodgkin's lymphomas of intermediate grade:

prolymphocytic-lymphoblastic, nodular type;
prolymphocytic, diffuse type;
prolymphocytic-lymphoblastic, diffuse type.

High-grade non-Hodgkin's lymphomas:

immunoblastic, diffuse type;
lymphoblastic (macro-, micro-, with twisted and non-twisted nucleus), diffuse type;
Burkitt's tumor.

Mycosis fungoides, reticulosarcomas (according to modern concepts, most of the latter are represented by lymphoid tumors and a minor part by the histiocytic variant), plasmacytoma, and unclassified lymphomas are allocated to separate sections of the WHO classification.

For nodular lymphosarcoma characteristic is the formation of pseudofollicular structures, which, unlike true follicles, are determined both in the cortical and medulla of the lymph node, are large, fuzzy contours and relatively monomorphic cellular composition.

Diffuse type of growth is characteristic of all variants of non-Hodgkin's lymphomas. Diffuse lymphocytic lymphosarcoma characterized by a total proliferation of cells such as small lymphocytes that infiltrate the walls of blood vessels, which leads to the complete erasure of the normal pattern of the lymph node. Such changes are similar to those detected in CLL, and therefore a complex of clinical and hematological signs (time parameters, localization of the tumor process, picture of peripheral blood, bone marrow, etc.) is of decisive importance in differential diagnosis.

Diffuse lymphoplasmacytic lymphosarcoma the combined proliferation of lymphoid and plasma cells is characteristic; plasmatized lymphocytes are also found. Changes in this variant of lymphosarcoma are similar to the picture that occurs with Waldenstrom's macroglobulinemia; the disease is often combined with various types of monoclonal gammopathy.

Diffuse prolymphocytic lymphosarcoma is characterized by the proliferation of cells larger than small lymphocytes, having round or irregularly shaped nuclei (“dissected” nuclei), in which 1-2 nucleoli are visible. The chromatin of the nucleus is less dense than that of a small lymphocyte. When the process is generalized, peripheral lymph nodes, liver, spleen, and bone marrow are most often affected (in 25-45% of cases). The five-year survival rate is 63-70%. Modern treatment ensures the practical recovery of most of the patients in the first stage of the process.

With diffuse lymphoblastic lymphosarcoma, proliferation of lymphoblast-type cells is found, among which macro- and microgenerations occur. Cells with nuclei of a brain-shaped (twisted, convolute) shape can be detected. They are more often observed in children with localization of the process in the lymph nodes of the mediastinum and, as a rule, have a T-cell nature. Lymphoblastic lymphosarcoma is characterized by the presence of a large number of cells in a state of mitosis, decaying cells.

Diffuse immunoblastic lymphosarcoma differs in massive growth of large mononuclear or multinuclear cells with a large centrally located nucleolus and an abundant zone of basophilic cytoplasm. A large number of mitoses, dying cells are revealed. Along with immunoblasts, a significant number of plasma cells are found. The prognosis is unfavorable, the five-year survival rate of patients ranges from 21 to 32%.

Burkitt's lymphoma differs by monomorphic proliferation of blast cells of the lymphoid type with hyperbasophilic often vacuolated narrow cytoplasm. Against this background, a typical, albeit non-specific, feature is the presence of large macrophages, which create a picture of a “driving sky”. There is an opinion about the proximity of Burkitt's lymphoma cells to partially blast-transformed B-lymphocytes. Unlike other forms of non-Hodgkin's lymphomas, the tumor is primarily localized mainly

extranodally.

At reticulosarcoma(histiocytic lymphoma), a relatively rare tumor, cell proliferation with morphological and functional features of macrophages, large cells of a round or elongated shape, containing a light, medium-sized bean-shaped nucleus with 1-2 nucleoli, surrounded by a rather wide rim of weakly basophilic cytoplasm, are found. Some cells show the ability to phagocytosis. Cells are characterized by high activity of nonspecific esterase, the ability to secrete lysozyme, and the absence of specific markers.

undifferentiated the type is characterized by the proliferation of sharply anaplastic cells with a large, irregularly shaped nucleus surrounded by a narrow zone of weakly basophilic cytoplasm. It is believed that some of these tumors are of lymphoid origin.

Along with the above classification, others are used. So, some authors suggest subdividing lymphosarcomas depending on the primary localization of the process; the term "lymphocytoma" emphasizes the benign course of extramedullary tumors, consisting mainly of mature forms of small lymphocytes (or lymphocytes and prolymphocytes), forming a structure of nodular growth. Therefore, they are isolated from low-grade non-Hodgkin's lymphomas into a separate group of lymphoid tumors.

The progression of non-Hodgkin's lymphomas may be accompanied by a change in the morphological variant of the disease, the transformation of nodular lymphosarcoma into diffuse.

Symptoms of Non-Hodgkin's Lymphoma

For all morphological variants of non-Hodgkin's lymphomas, there is an equally frequent lesion of both the lymph nodes in general and their individual groups, the Waldeyer lymphoid ring and the gastrointestinal tract. A more frequent primary lesion of the retroperitoneal lymph nodes and the abdominal cavity, bones and soft tissues is observed with lymphoblastic, spleen - with prolymphocytic variants. The pathological process, regardless of the morphological variant of the disease, in most cases first spreads not to the zones adjacent to the lymph nodes. The defeat of adjacent groups of lymph nodes often occurs in the lymphoblastic variant.

Early extranodal metastasis, bone marrow metastasis, involvement of the liver and spleen in the pathological process are somewhat more common in the prolymphocytic variant, and bone marrow damage and leukemization are more common in the presence of cells with a rounded and split nucleus. At the same time, in blast variants, the involvement of the bone marrow and the increase in the size of the lymph nodes occur earlier.

The greatest differences between morphological variants are noted when assessing survival. The five-year survival rate for the prolymphocytic variant of small cells with split and round nuclei is 70 and 53%, respectively. In the prolymphocytic-lymphoblastic variant of large cells with a split nucleus, the survival rates approach those in blast variants and are 14-21 months.

Survival rates in stages I-II of non-Hodgkin's lymphomas with a high degree of malignancy in the primary lesion of the gastrointestinal tract significantly exceed those observed in the general group of patients with these variants.

Primary non-Hodgkin's lymphoma of the spleen- rare localization (less than 1 % ), while its involvement in the pathological process is often (40-50%) found in lymphosarcomas. Somewhat more often, the primary lesion of the spleen is found in the prolymphocytic variant. More often, with spleen lymphoma, bone marrow is involved in the pathological process. However, in the lymphoblastic variant, metastases from the spleen are more often localized in the abdominal lymph nodes.

The most common lung involvement is found in low-grade non-Hodgkin's lymphomas. The prognosis for this primary localization is also determined by the morphological variant. The defeat of the nervous system ascertain, as a rule, with blast variants of non-Hodgkin's lymphomas.

The nodular type of non-Hodgkin's lymphomas within any histological type is characterized by a more favorable course of the disease. In the lymphocytic variant, despite the rapid generalization of the process, a relatively benign course is also noted.

The clinical and hematological picture in certain morphological variants of diffuse lymphosarcomas has its own characteristics. Yes, for lymphocytic variant a rather early generalization of the process is characteristic. Unlike chronic lymphocytic leukemia, it is often possible to trace the sequence of involvement and the pathological process of various groups of lymph nodes; histological examination of the bone marrow reveals a nodular or nodular-diffuse type of lesion (and unlike the diffuse nature of infiltration in chronic lymphocytic leukemia).

Generalization of the process, on average, occurs after 3-24 months. Bone marrow damage can also be detected with a normal hemogram (in 47% of patients it is not changed at the time of diagnosis), in some patients lymphocytopenia is detected. Despite the early generalization and involvement of the bone marrow in the process, the prognosis of the disease in this variant is relatively favorable (up to 75% of patients live for more than 5 years).

Clinical and hematological peculiarity differs T-cell variant of lymphosarcoma: splenomegaly, generalized enlargement of lymph nodes, infiltrates in the lungs, skin lesions. The primary focus is a T-dependent paracortical region of the lymph nodes. There is a high lymphocytosis in the blood, the nuclei of most of the lymphocytes are twisted. The average life expectancy in this rare variant is short - 10 months.

With a rare lymphoplasmacytic cytological variant, the clinical syndromes of the course of the disease are determined by the localization of the tumor, the degree of prevalence of the process, and often by the amount of IgM in the blood serum.

Prolymphocytic variant found in 45-51% of all cases of lymphosarcomas. With it, an increase in the occipital, parotid, popliteal, and lymph nodes is often detected. Despite the uneven generalization and frequent leukemization (in 25-45%) of the process, with this option, the five-year survival rate of patients is 63-70%. In the prolymphocyte-lymphoblastic subvariant, the prognosis is less favorable.

lymphoblastic variant, rather heterogeneous in its morphological (twisted, non-twisted nucleus, macro-, microforms) and immunological (T- and B-phenotype) characteristics, most often occurs in children. Lymph nodes of various localization are affected. The disease is distinguished by the rapid growth of tumors and the involvement of new anatomical zones in the process. More often than in other lymphosarcomas, the initial cytopenia, T-cell phenotype of lymphocytes, is found in the hemogram.

Burkitt's lymphoma of B-cell origin is attributed to the lymphoblastic type of lymphosarcoma. Its classic variant is manifested mainly by damage to the bones (especially the lower jaw), kidneys, ovaries, lymph nodes of the retroperitoneal regions, lungs, parotid salivary glands. The bone marrow is rarely involved in the process. With localized forms, the prognosis is favorable with long-term remissions up to a complete cure. The most common type of T-lymphoblastic lymphoma is "prothymocyte". In the vast majority of cases, the mediastinum is affected, metastases are detected in the central nervous system, lungs; in 50% of cases - leukemization. The disease is more often detected in boys of the first 5 years of life and adolescents 13-16 years old.

Immunoblastic lymphosarcoma(B-cell phenotype predominates) can develop as a primary tumor of the gastrointestinal tract, lymph nodes, Waldeyer rings, etc. Cytopenia is often detected, leukemization - in rare cases. The disease progresses rapidly, the five-year survival rate of patients is 21-32%, however, removal of a solitary tumor can contribute to many years of remission and even cure. Immunoblastic lymphosarcoma as a secondary process is described in multiple myeloma, Waldenström's macroglobulinemia, and other lymphoproliferative diseases.

Fungal mycosis is a malignant lymphoid tumor that always occurs primarily in the upper layers of the dermis, consisting of polymorphic T-helpers. The first manifestation of the disease may be nonspecific inflammation. The diagnosis is verified according to histological, cytochemical studies (lymphoid cells give a positive reaction to acid phosphatase, beta-glucuronidase and nonspecific acid esterase). There is a point of view that the early, chronic phase of the disease may be reactive, and the "lymphoblastic" represents a true malignant transformation. Cesari's syndrome, characterized by the appearance in the hemogram of lymphoid cells with a brain-shaped nucleus, is considered as the leukemic phase of mycosis fungoides.

The histiocytic variant of malignant non-Hodgkin's lymphomas is rare. Its clinical picture is varied. Metastases can be found in many organs. Leukemization and bone marrow involvement are rare, with cytopenia often present.

The nosological affiliation of the identified new forms remains debatable. So, Lennert's lymphoma, originally described as an unusual variant of lymphogranulomatosis with a high content of epithelioid cells, is proposed to be considered an independent form. The absence of typical Berezovsky-Sternberg cells, fibrosis, a high content of immunoblasts, plasma cells, transitions to lymphosarcoma served as the basis for distinguishing this disease from lymphogranulomatosis and isolating it under the name "Lennert's lymphoma" (malignant lymphoma with a high content of epithelioid histiocytes, lymphoepithelial lymphoma, epithelioid cell lymphoma ). A feature of the clinical manifestations of Lennert's lymphoma is the frequent defeat of the palatine tonsils of the lymph nodes, the elderly age of patients, the presence of polyclonal gammopathy and allergic skin rashes in history.

It is proposed to refer to non-Hodgkin's lymphomas also described in recent years angioimmunoblastic lymphadenopathy with dysproteinemia(lymphogranulomatosis X). Clinically, the disease is manifested by fever, weight loss, skin rashes, generalized lymphadenopathy, often in combination with hepato- and splenomegaly, persistent hyperglobulinemia, and sometimes signs of hemolysis. Histologically, a triad is characteristic: proliferation of small vessels, proliferation of immunoblasts, deposits of PAS-positive amorphous masses in the walls of blood vessels. The number of eosinophils and histiocytes fluctuates, but sometimes the number of the latter is markedly increased. Perhaps the presence of giant cells, small foci of necrosis. A number of researchers regard the changes described above not as malignant lymphoma, but as reactive, associated with disorders in the B-lymphocyte system.

Lymphocytes can be localized in various organs and tissues (spleen, lymph nodes, stomach, lungs, skin, etc.). The disease progresses slowly. For a long time, the spleen is slightly enlarged, the lymph nodes are of normal size or slightly enlarged. In the blood, the number of leukocytes is normal or close to normal, with a predominance or normal content of mature lymphocytes. The level of platelets is within the normal range, their number may decrease to 1*10 9 /l-1.4*10 9 /l in some patients after 7-10 years. More often, only a slight tendency to a decrease in the level of hemoglobin and the number of erythrocytes is revealed, reticulocytes fluctuate within 1.5-2%. Bone marrow biopsy reveals individual proliferates consisting of mature lymphocytes; histological studies of an enlarged lymph node and other affected organs help to verify the diagnosis. Malignancy of a lymphocytoma with transformation into lymphosarcoma or chronic lymphocytic leukemia is not mandatory, and if it does occur, it is often after many months or years.

Diagnosis of Non-Hodgkin's Lymphoma

The first symptoms of lymphosarcoma are an increase in one (49.5%) or two (15%) groups of lymph nodes, generalized adenopathy (12%), signs of intoxication, leukocytosis (7.5%) or leukocytopenia (12%) in the hemogram, lymphocytosis ( 18%), increased ESR (13.5%). Differential diagnosis should be carried out with chronic lymphocytic leukemia, infectious mononucleosis, nonspecific lymphadenopathy. From the period of the appearance of the first signs of the disease to the establishment of a true diagnosis, months often pass.

Primary extranodal lesions can occur in any organ where there is lymphoid tissue. Only 15 cases of isolated liver damage have been described, but its metastatic lesion is found in more than 50% of patients. The primary localization of the process in the spleen (less than 1%), mammary gland, lungs and pleura is rare.

The diagnosis of lymphosarcoma is established on the basis of data from a histological examination of the lymph nodes or other tumor formations, their cytological (imprint, punctate), cytochemical and immunological studies are mandatory. For diagnostic purposes and to assess the prevalence of the pathological process, punctate and biopsy of the bone marrow are examined.

Lymphocytomas proceed favorably for a long time. Peripheral lymph nodes are often slightly enlarged, the spleen in the splenic variant is large, a low content of lymphocytes in the blood and their focal proliferation in the bone marrow are found. The tumor substrate is predominantly composed of mature lymphocytes (or lymphocytes and prolymphocytes), which form a structure of nodular growth. After a long time, the transformation of lymphocytes into lymphosarcomas or chronic lymphocytic leukemia is possible.

Treatment for non-Hodgkin's lymphoma

Therapy for lymphosarcoma is determined primarily by the morphological variant (degree of malignancy), the nature of the prevalence of the pathological process (stage), the size and location of the tumor, the age of the patient, the presence of other diseases.

The fundamental method of treatment of non-Hodgkin's lymphomas is polychemotherapy, which can ensure the development of clinical and hematological remission, its consolidation and anti-relapse treatment. Local radiation therapy in most cases is justified in combination with chemotherapy or as a palliative irradiation of tumor formations. As an independent method, radiation therapy for non-Hodgkin's lymphomas can be used only in clearly proven stage I of the disease in low-grade non-Hodgkin's lymphomas, with bone involvement in the process.

After local radiation therapy or surgical removal of the tumor, complete remission persists in many patients for more than 5-10 years. The disease progresses slowly with the generalization of the process.

Elderly patients with low-grade non-Hodgkin's lymphomas, as well as in the presence of severe concomitant diseases, can be treated with monochemotherapy (chlorbutin, pafencil, cyclophosphamide, etc.). With a primary lesion of the spleen, regardless of the morphological variant of the disease, a splenectomy is performed followed by a course of chemotherapy. With an isolated lesion of the stomach, its resection is included in the combined treatment program. For skin lesions, chemotherapy is used, including prospidin and spirobromine.

In stages III-IV of the disease in aggressive non-Hodgkin's lymphomas, induction of remission is carried out by courses of polychemotherapy followed by consolidating treatment. On the area of the most aggressive tumor growth, local radiation therapy can be used, it is also effective in primary lymphosarcomas of the pharyngeal ring. However, since the possibility of a generalization of the process cannot be ruled out, it is advisable to combine radiation treatment with chemotherapy.

In generalized stages of non-Hodgkin's lymphomas of low grade malignancy, the programs COP, COPPP, BACOP, etc. are used.

With blast variants, it is advisable for young people to prescribe programs for the treatment of acute lymphoblastic leukemia and the prevention of neuroleukemia.

The results of cytostatic therapy for non-Hodgkin's lymphomas also depend on the timely use of hematological resuscitation, immunocorrection and antibiotic therapy.

Since hematopoiesis in some patients remains intact for some time, it is possible to conduct polychemotherapy on an outpatient basis, which, with a clear organization of dispensary observation, facilitates the treatment process and allows patients to avoid the severe psychological effects of oncological and hematological hospitals.

After achieving clinical and hematological remission for 2 years at intervals of 3 months. anti-relapse therapy. Upon reaching a stable remission, treatment is stopped.

In the last 10 years, survival in aggressive non-Hodgkin's lymphomas has increased markedly due to the use of programs that include 5-6 cytotoxic drugs. Modern therapy contributes to the achievement of complete remissions in 70-80% and 5-year relapse-free survival in 65-70% of patients.

In recent years, clinical experience has been accumulating in the use of allogeneic and autologous bone marrow transplants for non-Hodgkin's lymphomas.

Lymphocytomas may not require special treatment for a long time. In their therapy, surgical removal of the tumor, monochemotherapy (cyclophosphamide, chlorbutine), glucocorticosteroids, antihistamines and other agents are used according to indications. In cases of transformation into lymphosarcoma or chronic lymphocytic leukemia, combined cytostatic therapy programs are prescribed for these diseases.

Forecast disease depends on the type of lymphoma, the stage of the disease, its spread, response to treatment, etc. Properly selected treatment increases the prognosis for recovery.

Prevention of non-Hodgkin's lymphoma

To date, the method by which it would be possible to prevent the occurrence of non-Hodgkin's lymphoma is unknown. Most people with non-Hodgkin's lymphoma have not been exposed to known risk factors.

Lymphoma A type of cancer involving cells of the immune system called lymphocytes. Lymphoma is found in many different types of cancer (about 35). But all diseases of this type are divided into two categories:

Non-Hodgkin's lymphomas.

The first type affects only about 12% of people and is currently a curable disease.

Non-Hodgkin's cancer attacks the body's lymphatic system, which is made up of blood vessels that carry a fluid called "lymph" to the body. When subtypes of B or T cells begin to multiply uncontrollably, a malignant transformation is formed. The abnormal cells may collect in one or more lymph nodes or other tissues such as the spleen. Through the lymphatic system, the tumor quickly spreads to distant parts of the body.

Fast-growing lymphoma - prognosis

There is an international prognostic index that has been developed to help doctors determine the outlook for people with fast-growing lymphoma as well.

The indicator depends on 5 factors:

The patient's age.
Lymphoma stages.
The presence of a disease in the organs of the external lymphatic system.
General condition (how well a person can perform daily activities).
A blood test to determine the level of lactate dehydrogenase (LDH), which comes with the amount of lymph.

Many conditions affect the positive prognosis for lymphoma:

patient's age (up to 60);
stage (I or II);
the absence of lymphoma outside the lymph nodes or the presence of metastases in only one area outside them;
the patient's body is able to function normally;
serum LDH is normal.

If the indicators differ from those indicated, this indicates poor prognostic factors. Accordingly, on a 5-point scale, such patients have the least comforting prognosis for recovery and survival. In this regard, there are 4 risk groups:

Low (zero or single adverse circumstances).
Low intermediate (2 adverse conditions).
Cancer diseases with a high intermediate level (3 low survival rates).
High (4 or 5 adverse factors).

Studies developed over the past decade indicate that about 75% of people in the lowest risk group lived at least 5 years. While in the high risk group, about 30% of patients lived for 5 years.

In the last five years, treatment methods have changed, and new procedures have been developed for the treatment of malignant tumors, which positively affects prognostic data. World studies in recent years show that about 95% of people in the very low risk group lived at least 4 years. While at high levels of danger (low survival), life expectancy is already 55%.

Follicular lymphoma - survival prognosis

For follicular lymphoma, which is characterized by slow growth, other prognostic indices have been developed.

Good predictive factors:

age under 60;
I or II stages;
hemoglobin 12/g or higher;
metastatic lesion has spread to 4 or fewer lymphatic areas;
serum LDH is normal.

Risk groups and survival:

Low risk (1 adverse factor): 5-year survival becomes 91%, 10-year ‒ 71%.
Intermediate risk (2 adverse factors): 5-year survival - 78%, 10-year - 51%.
High risk (3 negative factors): 5-year survival - 53%, 10-year - 36%.

Survival in lymphoma

The survival rate directly depends on the stage of the oncological disease. Also, it should be taken into account that modern clinical trials (for example, such as stem cell transplantation) can reduce the risk of an adverse outcome.

Survival also varies greatly by subtype of non-Hodgkin's lymphoma. Follicular lymphoma has a better prognosis (96%) than diffuse B-cell lymphoma (65%). characterized by readings that become 71%. For the marginal zone of the disease - 92%.

Lymphatic cancer with a survival prognosis by stage is presented as follows:

In patients with stage 1 lymphoma, five-year prognostic data are quite high - 82%.
Patients with the second stage of the disease have an 88% five-year survival rate.
The five-year prognosis from the initial detection of the disease for patients of the third stage includes 63%.
The fourth stage of oncological lesions is represented by 49% for 5 years of the course of the disease from the first diagnosis.

Five-year survival for young men and women is the highest and decreases with increasing age:

in men, the five-year prognosis for lymphoma ranges from 83% (for 15–39 year olds) to 36% (for 80–99 year olds);
in women, the five-year survival rate for lymphoma becomes 86% to 40% in the same age groups.

Compared to the 1990s, in 2014-2015 cancer of the lymphatic system in most cases can have a favorable outcome. Thus, the five-year standardized survival rate in men increased by 30%, and in women by 39 points, which represents a positive trend.

14.10.2017

Malignant tumor processes of the lymphoproliferative type, or non-Hodgkin's lymphomas, can develop in different ways and respond to drugs in an unpredictable way. This type of tumor is characterized by rapid development, which is why the prognosis for the life of patients with non-Hodgkin's lymphoma is not clear.

Malignant types of lymphatic tumor lesions are formed in the lymphoid tissues. The main sign by which non-Hodgkin's lymphoma is determined is the lack of response to the methods of treating Hodgkin's lymphomas. A separate characteristic factor is germination in organs that are not part of the lymphatic system. In the process of development of pathology, prediction is impossible.

The prognosis of non-Hodgkin's lymphoma for a patient depends on a combination of factors:

the result of histological examination;
current stage of tumor development;
conformity of therapeutic methods.

The tumor itself has properties that differ from other malignant neoplasms in morphological terms, clinical manifestations and development prognosis. Since 1982, non-Hodgkin's lymphoma has been called non-Hodgkin's lymphoma in accordance with the accepted classification; this type of tumors is divided into three large groups according to the degree of aggressiveness of development.

The comparative prevalence of NHL is high - in relation to all types of lymphomas, non-Hodgkin's develops in 88% of cases. And if you look at the statistics as a whole, then the occurrence of lymphomas in comparison with the total number of malignant neoplasms is about 4% of which 12% are Hodgkin's.

Reasons for development

The formation of this type of lymphatic tumor comes from T cells, unlike the rest. Specialists have not identified immediate causes for the development of non-Hodgkin's lymphosarcoma, but some provoking factors are indicated:

radiation and chemotherapy;
organ transplantation;
exposure to carcinogens;
autoimmune thyroiditis;
severe viral infections.

Gradually, their development provokes a systemic failure and the beginning of the growth of a tumor neoplasm. In the first period, the process can take place completely without any manifestations. With further progression of the tumor formation, certain symptomatic manifestations are possible, the occurrence of which is a clear reason to contact medical specialists.

Symptoms

The main sign of a developing lymphatic neoplasm is an increase in lymph nodes with increased soreness. Mostly such manifestations become noticeable in the areas of the groin, neck, armpits. The secondary symptoms are:

significant weight loss;
increased sweating during sleep;
chronic fatigue;
itching of the skin of the whole body;
feverish conditions;
signs of anemia.

Some types of non-Hodgkin's lymphoma have distinct symptoms.

Lymphoblastic

Lymphoblastic lymphoma is characterized by the development of lesions in the abdominal cavity. Pathology progresses latently, all symptoms are erased, which is why detection occurs in the later stages and it is impossible to achieve a complete cure. Active metastasis of lymphoblastic lymphoma affects nearby organs:

liver;
spleen;
ovaries;
kidneys;
spinal cord.

At the terminal stage of the disease, neuro-systemic paralysis occurs, which leads to additional damage to the body. The only way to treat is to inhibit the development of the neoplasm and introduce the tumor into stable regression.

Lymphocytic

Lymphocytic neoplasms consist of fully mature b-type cells. May be characterized by localized or generalized swelling of the lymph nodes and painlessness. It most often occurs in older patients and can affect the following organs:

spleen;
liver;
lungs;
bone structures.

Most often, diagnosis occurs at stage 4 of tumor development with damage to bone marrow structures and tissues.

Intestinal lymphoma

Nihodgkin's lymphoma of the gastrointestinal tract is more often extranodal in development, and the process itself is a secondary development after metastasis. Among the symptoms of pathology note:

pain in the abdominal region;
flatulence;
enlargement of the spleen;
gagging;
intestinal obstruction;
loss of appetite;
admixture of blood in faeces.

Most often, the development of the disease begins against the background of HIV, Crohn's disease or blood protein deficiency.

Non-Hodgkin's lymphoma of the spleen

This type of neoplasm occurs more often in the elderly and begins in the marginal part of the spleen. At the beginning of the development of pathology, there are no symptoms, but with progression they are expressed as:

fever in the evening;
sweating during sleep;
rapid weight loss;
rapid satiety during meals;
heaviness in the hypochondrium on the right;
anemia.

For lymphoma of the spleen, extronodality is characteristic, which causes a pronounced pain syndrome. Gradually, the process is transferred to the bone marrow structures. A significant increase in the spleen in the later stages of neoplasm development is the most characteristic sign of this type of pathology.

Diagnosis of pathology

If a lymphatic neoplasm is suspected, it is necessary to confirm the development of the tumor process with analyzes and hardware studies.
Diagnosis requires the appointment of procedures:

laparoscopy;
biopsy of lymph nodes;
ultrasound procedure;
thoracoscopy;
lymphoscintigraphy;
tomography (MRI or CT);
bone marrow puncture.

Depending on the results, oncohematologists can draw a conclusion about the type of tumor neoplasm. This allows an approximate assessment of the extent and stage of the lesion, as well as acceptable treatment options.

Types of lymphosarcomas

There are several types of cellular structures of the lymphatic system, and each of them has its own characteristics. The formation of a lymphatic tumor can begin on the basis of each of them and will have separate characteristics.
B-cell neoplasms are characterized by an increased degree of aggressiveness and rapid extranodal (with the transition to other organs) spread. They are divided into:

Large cell. They are characterized by a clear increase in the axillary and cervical nodes of the lymphatic system.
Follicular. There is persistent redness, soreness and swelling of the inguinal, cervical and axillary zones of the lymphatic system.
Diffuse large cells. There are specific plaque-shaped rashes and ulcers on the skin.
marginal lymphosarcoma. It is characterized by an increase in pain in the area of occurrence, can develop hidden and is most often located in the peritoneum.

T-cell types of neoplasms in the lymphatic system usually affect the skin and lymph nodes. In males, this type of NHL occurs more frequently. Type t lymphomas include:

peripheral;
skin;
t-lymphoblastic;
t-cell angioimmunoblastic.

T/NK cell neoplasms are classified as aggressive lymphosarcomas. The disease develops rapidly and its progression is unpredictable, and the ability to live 5 years is approximately 33% of cases.

medical classification

There are several types of classification of non-Hodgkin's lymphomas. Each of the groups must be associated with some parameter. According to the rate of pathological progression, there are:

indolent;
aggressive;
highly aggressive.

According to the histological examination, determine:

reticulosarcomas;
lymphosarcomas;
lymphoblastomas of the t-cell type;
diffuse type lymphosarcomas;
primary lymphomas of the nervous system;
follicular lymphomas;
nodular neoplasms;
Burkitt's lymphoma, etc.

The localization parameter of development implies nodal neoplasms (limited to damage to the lymphatic system) and extranodal (the developing tumor is transferred to other organs and systems of the body). According to cytological indicators, small-cell and large-cell neoplasms are distinguished.

Treatment, stages and prognosis

The combination of different directions of influence on the disease gives the highest results. In the case of abnormal development, the treatment of NHL lymphoma allows the use of polychemotherapy. The technique is based on the use of several groups of chemotherapy drugs, as a result, a stable remission occurs.

Treating older patients with non-Hodgkin's lymphoma presents its own challenges. According to statistical indicators - 65% of complete remissions are achieved in patients under the age of forty, in the older group this value drops to 37% with an increase in the number of deaths to 30% of cases. In 1996, the methodology for the use of chemotherapy drugs was revised - the period was extended to 8 days with the division of dosages of Doxorubicin and Cyclophosphamide with the use on the first and eighth days.

After determining the type of pathology detected in the patient, the doctor must determine the degree of damage and prescribe a scheme for influencing the neoplasm. The classic treatment options for non-Hodgkin's lymphoma are:

With stage 1-2 lymphoma, it is recommended to prescribe a course of chemotherapy. It is possible to increase the chances of tumor regression by parallel with the course of chemotherapy, stem cell transplantation.
Stage 3-4 NHL requires chemotherapy. The main goal of such treatment is to bring the disease into a state of long-term remission.
The use of radiation therapy may be prescribed in cases of lesions of the t-cell form of non-Hodgkin's lymphoma. The onset of remission can last several years.
Alternative methods of treatment are allowed - immunotherapy, hormone therapy or stem cell and bone marrow transplantation.

Alternative methods quite often contribute to an increase in internal potential and increase the duration of remission. Surgery can also be applied to non-Hodgkin's lymphoma, but subject to a number of conditions:

the tumor is localized;
has a low degree of malignancy;
additionally, immune preparations are used.

Radiation therapy is used for isolated tumors or in cases where chemotherapy is not possible or contraindicated.

stages

When considering non-Hodgkin's lymphoma in terms of stages of development, there are 4 main ones in accordance with the ICD (International Classification of Diseases):

At the initial stage of NHL development, symptoms are low, and the neoplasm is represented by a single lesion, which is localized in the lymph nodes.
The second stage of tumor development implies an extranodal type of multiple organ damage or the simultaneous development of two or more tumors in the lymphatic system.
The third stage of NHL involves the development of neoplasms in the retrosternal region and their spread to the peritoneal organs.
The fourth is the terminal stage of the development of the tumor process. It involves metastasis of lymphoma to the main systems of the body (nervous system, bone marrow and musculoskeletal system).

It should be noted that in the terminal stage, the initial localization of the disease does not matter to determine the form of treatment and the degree of risk.

Forecast for life

In a classic situation, with the development of a lymphatic tumor, the period of life of patients is calculated on a special scale. Each of the negative signs of the disease is equal to 1 point, and after their determination, a general calculation takes place:

A favorable prognosis is considered when the NHL assigns no more than 2 points.
If the signs are from 2 to 3, the forecast is considered uncertain.
When the scores rise to 3-5, it is considered that the prognosis is negative.

One of the factors that are taken into account in the calculation is the frequency of recurrence of pathology. More often, favorable prognosis is given to patients with a b-cell type of tumor, and this is rare with a t-cell type.
For an approximate prediction of life with non-Hodgkin's type of lymphoma, there is a statistical table for a five-year period:

Summary statistics for determining the risks of 5-year survival in non-Hodgkin's lymphoma.
Type of lymphoma	Average	Percentage of patients surviving 5 years
Type of lymphoma	Average	0-1 factor	4-5 factors
Follicular	75	83	12
mantle zone	29	58	12
Peripheral t-cell	26	64	15
	45	72	22
marginal zone	70	90	52
Anaplastic large cell	77	80	77

For aggressive types of lesions with a patient under 60 years of age, a separate method is more often used with calculations based on the presence of three unfavorable factors:

3-4 stages of neoplasm development;
increased lactate dehydrogenase (LDH);
increased score on WHO-ECOG (from 2 to 4).

The amount of risk in this option is subdivided according to the following distribution:

Low risk category, implying the complete absence of the listed factors. Survival for a period of 5 years or more in 83% of cases.
A category of low intermediate risk, which implies the presence of no more than one factor. The survival rate is about 69% of cases.
The category of increased intermediate risk implies the presence of 2 provoking factors. Survival in a 5-year period is on average 46% of cases.
High-risk category, meaning the presence of three factors from the ECOG list. The average survival rate for 5 years or more in 32% of cases.

Positive forecasts for a period of 5 years are forecasts for neoplasms of NHL in the areas of glandular tissues and the abdominal cavity. Negative prognoses are more often made when the tumor is localized in the glandular tissues of the breast, ovaries, nervous tissue and bone tissue.

Oncological diseases are today one of the most severe and difficult to treat diseases. These include non-Hodgkin's lymphoma. However, there are always chances, and a clear idea of what the disease is, its types, causes, methods of diagnosis, symptoms, methods of treatment and prognosis for the future can increase them.

What is a disease

To understand what non-Hodgkin's lymphoma is, one should understand the concept of lymphoma in general. Lymphoma is the name that unites a group of oncological diseases. Cancer cells affect the lymphatic tissue, the lymph nodes greatly increase in size, somatic organs may be affected. Non-Hodgkin's lymphoma is any type of disease that is not Hodgkin's lymphoma.

What can cause the disease

The causes of non-Hodgkin's lymphoma are not yet fully understood. Indirectly, the following factors can affect the development of the disease and further prognosis:

work with aggressive chemicals;
living in areas with unfavorable environmental conditions;
severe viral infections, in particular the Epstein-Barr virus;
exposure to ionizing radiation.

It can also be noted that men suffer from non-Hodgkin's lymphoma somewhat more often than women. Also, the probability of detecting the disease increases with age: according to statistics, among the sick, the majority are people over 40 years old. The form of the disease often depends on age: children and young people are more likely to have symptoms of a small cell form of the disease or Burkitt's lymphoma, and follicular lymphomas are usually observed in the elderly.

Classification of different forms of the disease

As already mentioned, non-Hodgkin's lymphoma is a name that unites a group of similar (quite similar symptoms, treatments and prognosis for the future), but still different diseases. And these diseases can be divided according to the following features:

The nature of the course of the disease

On this basis, non-Hodgkin's lymphomas are divided into two types: aggressive and indolent lymphomas, which progress rapidly, literally "gush" with various symptoms and spread throughout the body at an incredible speed. And oddly enough, it is aggressive lymphomas that are most likely to be cured. Indolent lymphomas are characterized by a sluggish, chronic course with sudden relapses. It is these relapses that often cause the death of the patient. In addition, indolent-type lymphoma tends to degenerate into diffuse large cell, which will significantly increase malignancy and, accordingly, worsen the prognosis.

Tumor localization

Although the name "lymphoma" suggests primarily the defeat of the lymph nodes, lymphoma can be found not only there. Depending on the localization, the following types of lymphomas are distinguished:

Nodal. In this variant, the tumors are located exclusively in the lymph nodes. As a rule, we are talking about the initial stages of the disease. The prognosis for this form is quite good, the chance of a long-term remission is high.
Extranodal. In this case, lymphoma spreads to tissues and organs - through lymph or blood. In more advanced stages of the disease, tumors invade the bones and brain. One of the most severe forms of extranodal lymphomas is Burkitt's lymphoma.
diffuse. In this case, it is most difficult to detect lymphoma, since pathogenic cells are located on the walls of blood vessels, therefore the pattern of the lymph node is erased. According to the shape of the cells, diffuse lymphoma can be: polylymphocytic (in this case, large round pathogenic cells are observed), lymphoblastic (twisted cells), immunoblastic (there is a strong proliferation of cells around the central cell - nucleoli) and not differentiable.

Symptoms of the disease

As a rule, the earliest stages of the disease are almost asymptomatic. However, a little later, you can notice the following symptoms of the disease:

Severe enlargement of the lymph nodes (usually cervical and axillary).
Weakness, lethargy.
Elevated temperature.
Pretty fast weight loss.
Excessive sweating even in cool or cold weather.
Headache.

All of the above symptoms are quite common, so it is quite difficult to detect lymphoma in the early stages. However, if you still manage to find an enlarged lymph node, you should immediately contact a specialist, because an increase in lymph nodes can occur both from a common cold and from such a serious disease as lymphoma.

How is the diagnosis carried out

In addition to the collected anamnesis, a number of additional studies are needed to establish an accurate diagnosis. Among them:

Histological examination (puncture or biopsy of the lymph node). Thanks to this analysis, pathological cells can be detected in the lymph, indicating the presence of lymphoma.
Cytological and cytochemical analyzes based on punctate.
Immunological research.

When it is possible to find out if a person has lymphoma, other studies are assigned to help establish all the parameters of the disease, because further treatment depends on it.

Stages of development of the disease

During the diagnosis of the disease, its malignancy and stage of the disease are determined. Malignancy can be high, low, or intermediate. As for the stages, there are four.

I stage

At the initial, first stage of the disease, only a separate inflamed lymph node can be observed, general symptoms (weakness, poor health, hyperthermia) are not observed.

II stage

In the second stage of a disease such as non-Hodgkin's lymphoma, the tumors become multiple. Gradually, general symptoms begin to appear. If we are talking about the B-cell form, then at this stage, neoplasms begin to be divided into those that are to be removed and those that are not to be removed.

III stage

At this stage of the development of the disease, tumors often spread to the diaphragm, abdominal cavity, and chest. Tissues are affected, as well as all or almost all internal organs.

IV stage

The last and most severe stage of the disease. The prognosis in this case, alas, is very disappointing. The disease affects not only the organs, but also the bone marrow, gets to the central nervous system and bones. If the treatment was delayed so much that the lymphoma passed into this stage, then there can be no talk of recovery.

What are the treatments for non-Hodgkin's lymphoma?

In many ways, the methods by which treatment will be carried out depend on the nature of the disease, the size of the tumors, the stage and malignancy. But in general, the following ways to combat the disease can be distinguished:

Chemotherapy. It is she who is the basis of treatment. In the early (first and second) stages of low-grade lymphomas, monochemotherapy is used. If the stages are severe or the lymphoma behaves extremely aggressively, then polychemotherapy is used. Sometimes chemotherapy is combined with radiation therapy, but irradiation in its pure form can only help at stage I. Also, radiation therapy is sometimes applied locally in those areas where the neoplasm behaves most aggressively.
Surgical removal of the tumor. Of course, this method is used only if the stage and type of tumor allows. With the successful use of radiation therapy and surgical intervention, the prognosis is quite positive: remission may occur for a period of 5 to 10 years.
The use of programs BATsOP, TsOP, etc. I must say that these programs show fairly good performance.
Palliative care designed to relieve pain and improve the patient's quality of life to the extent possible. It is used in the terminal stages, when the prognosis is unfavorable and the chances of defeating the disease are very small. In addition to medication, the patient may need the help of a psychologist, as well as the support of relatives.

What is the prognosis of the outcome of the disease

As already mentioned, the treatment, as well as the effect of it in non-Hodgkin's lymphoma, is a very individual matter. Someone manages to achieve a full recovery on the first try, someone has to face relapses, someone can only extend their life by several years, and in some cases medicine is completely powerless. However, the diagnosis should not be considered a verdict: with timely diagnosis, proper treatment and a not too problematic form of the disease, the chances of recovery reach 65-70%. And that's enough.

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