Prognosis for life with non-Hodgkin's lymphoma. Features of the clinical picture of non-Hodgkin lymphoma Non-Hodgkin 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 subsequently metastasize through the lymphogenous or hematogenous route. The clinical picture of lymphoma is characterized by lymphadenopathy, symptoms of damage to one or another organ, and fever-intoxication syndrome. Diagnosis is based on clinical and radiological data, the results of a hemogram, 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, lymphosarcoma) are malignant lymphoproliferative tumors of different morphology, clinical signs and course, different in their characteristics from Hodgkin's lymphoma (lymphogranulomatosis). Depending on the location of the primary focus, hemoblastoses are divided into leukemia (tumor lesions of the bone marrow) and lymphoma (tumors of lymphoid tissue with a primary extramarrow localization). Based on distinctive morphological features, lymphomas are in turn divided into Hodgkin and non-Hodgkin; The latter in hematology include B- and T-cell lymphomas. Non-Hodgkin's lymphomas occur in all age groups, but more than half of the cases of lymphosarcoma are diagnosed in people over 60 years of age. The average incidence rate among men is 2-7 cases, among women – 1-5 cases per 100,000 population. Over the past few years, there has been a trend toward a progressive increase in incidence.

Causes

The etiology of lymphosarcoma is not reliably known. 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 significant, while the contribution of others to the etiology of lymphomas is very insignificant. These types of unfavorable conditions include:

Infections. Human immunodeficiency virus (HIV), hepatitis C, and T-lymphotropic virus type 1 have the greatest cytopathogenic effect on lymphoid cells. A connection between Epstein-Barr virus infection and the development of Burkitt's lymphoma has been proven. It is known that Helicobacter pylori infection, associated with gastric ulcer, can cause the development of lymphoma of the same localization.
Immune defects. The risk of lymphoma increases with congenital and acquired immunodeficiencies (AIDS, Wiskott-Aldrich syndrome, Louis-Bar syndrome, 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 is observed among patients with rheumatoid arthritis, lupus erythematosus, which can be explained by both immune disorders and the use of immunosuppressive drugs to treat these conditions. Thyroid lymphoma usually develops against the background of autoimmune thyroiditis.
Toxic effects. A cause-and-effect relationship is traced between lymphosarcoma and previous contact with chemical carcinogens (benzene, insecticides, herbicides), UV radiation, and radiation therapy for cancer. Cytostatic drugs used for chemotherapy have a direct cytopathic effect.

Pathogenesis

Pathological lymphogenesis is initiated by one or another oncogenic event, causing disruption of the normal cell cycle. Two mechanisms may be involved in this - activation of oncogenes or suppression of tumor suppressors (antioncogenes). The tumor clone in NHL in 90% of cases is formed from B lymphocytes, extremely rarely from T lymphocytes, NK cells or undifferentiated cells. Various 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 bone marrow infiltration, cytopenia develops. The growth and metastasis of the tumor mass is accompanied by cachexia.

Classification

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

The modern classification includes over 30 different types of lymphosarcoma. 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 NHL number. The course is indolent, but transformation into aggressive diffuse lymphoma is possible. The 5-year survival rate is 60-70%.
small cell lymphocytic lymphoma and chronic lymphocytic leukemia– similar types of NHL, which account for 7% of their number. The course is slow, but difficult to treat. The prognosis is variable: in some cases, lymphosarcoma develops within 10 years, in others, at a certain stage it turns into a fast-growing lymphoma.
mantle cell lymphoma– in the NHL structure it is 6%. Only 20% of patients overcome the five-year survival threshold.
Marginal zone cell B-cell lymphomas– are divided into extranodal (can develop in the stomach, thyroid, salivary, mammary glands), nodal (develop in the lymph nodes), splenic (localized in the spleen). Characterized by slow local growth; in the early stages they are highly treatable.
B-cell mediastinal lymphoma– is rare (in 2% of cases), but unlike other types it affects mainly young women 30-40 years old. Due to rapid growth, it causes compression of the mediastinal organs; cured in 50% of cases.
Waldenström's macroglobulinemia(lymphoplasmacytic lymphoma) – diagnosed in 1% of patients with NHL. It is characterized by hyperproduction of IgM by tumor cells, which leads to increased blood viscosity, vascular thrombosis, and capillary ruptures. It can have both relatively benign (with survival 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 progression of the tumor is slow and does not always require 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 half of the patients to be cured.
lymphoma of the central nervous system– Primary damage to the central nervous system may affect the brain or spinal cord. More often associated with HIV infection. The five-year survival rate is 30%.

Non-Hodgkin lymphomas of T-cell origin are represented by:

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

Symptoms

The clinical manifestations of NHL vary greatly depending on the location of the primary lesion, the extent 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 enlargement of peripheral lymph nodes. At first they remain elastic and mobile, later they merge into vast conglomerates. The lymph nodes of one or many areas can be affected at the same time. When fistula tracts form, it is necessary to exclude actinomycosis and tuberculosis.

Nonspecific symptoms of lymphosarcoma such as fever without obvious causes, night sweats, weight loss, and asthenia in most cases indicate the generalized nature of the disease. Among extranodal lesions, non-Hodgkin lymphomas of the Pirogov-Waldeyer ring, gastrointestinal tract, and brain dominate; the mammary gland, bones, lung parenchyma, and other organs are less commonly affected. On endoscopic examination, nasopharyngeal lymphoma looks like a pale pink tumor with bumpy contours. Often the maxillary and ethmoid sinuses and orbit grow, causing difficulty in nasal breathing, rhinophony, hearing loss, and exophthalmos.

Primary testicular lymphosarcoma may have a smooth or lumpy surface, elastic or stony density. In some cases, swelling of the scrotum, ulceration of the skin over the tumor, and enlargement of the inguinal-iliac lymph nodes develop. Testicular lymphomas are prone to early dissemination with damage to 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 stomach cancer, accompanied by pain, nausea, loss of appetite, and weight loss. Abdominal lymphosarcoma can manifest itself as partial or complete intestinal obstruction, peritonitis, malabsorption syndrome, abdominal pain, ascites. Lymphoma of the skin is manifested by itching, nodules and a reddish-purple induration. Primary damage to 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. When the mediastinal lymph nodes are damaged, 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. Germination of the walls of the stomach or intestines is dangerous due to the occurrence of bleeding (in case of vascular arrosion) or peritonitis (when contents leak into 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

Diagnosis of non-Hodgkin lymphomas is the responsibility of oncohematologists. Clinical criteria for lymphosarcoma are enlargement of one or more groups of lymph nodes, intoxication phenomena, and extranodal lesions. To confirm the suspected diagnosis, it is necessary to carry out morphological verification of the tumor and instrumental diagnostics:

Study of tumor cell substrate. Diagnostic operations are performed: puncture or excisional biopsy of lymph nodes, laparoscopy, thoracoscopy, bone marrow aspiration puncture 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.
Visualization methods. Enlargement of the mediastinal and intra-abdominal lymph nodes is detected using ultrasound of the mediastinum, radiography and CT scan of the chest and abdominal cavity. The examination algorithm according to indications includes ultrasound of the lymph nodes, liver, spleen, mammary glands, thyroid gland, scrotal organs, gastroscopy. To stage the tumor, an MRI of internal organs is performed; Lymphoscintigraphy and bone scintigraphy are informative in identifying metastases.
Laboratory diagnostics. Aimed at assessing risk factors and internal organ function in lymphomas of various locations. In the risk group, the HIV antigen and anti-HCV are 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 unique tumor marker for NHL.

Chemotherapy. Most often, treatment of lymphomas begins with a course of polychemotherapy. This method can be used alone or combined with radiation therapy. Combined chemoradiation therapy allows for 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 treatment cycles.
Surgical interventions. It is usually used for isolated damage to any organ, more often the gastrointestinal tract. Whenever possible, operations are radical in nature - extended and combined resections are performed. In advanced cases, when there is a threat of perforation of hollow organs, bleeding, or intestinal obstruction, cytoreductive interventions can be performed. Surgical treatment is necessarily complemented by chemotherapy.
Radiation therapy. As a monotherapy for lymphomas, it is used only for localized forms and low-grade tumors. In addition, radiation can also be used as a palliative method if other treatment options are not possible.
Additional treatment regimens. Of the alternative methods, immunochemotherapy using interferon and monoclonal antibodies has proven itself to be successful. To consolidate remission, transplantation of autologous or allogeneic bone marrow and the introduction of peripheral stem cells are used.

Prognosis and prevention

The prognosis for non-Hodgkin lymphomas varies, depending mainly on the histological type of tumor and stage of detection. With locally advanced forms, long-term survival averages 50-60%, with generalized forms - only 10-15%. Unfavorable prognostic factors include age over 60 years, stages III-IV of the oncological process, bone marrow involvement, and the presence of several extranodal lesions. At the same time, modern PCT protocols in many cases make it possible to achieve long-term remission. Prevention of lymphomas correlates with known causes: it is recommended to avoid infection with cytopathogenic viruses, toxic effects, and excessive sun exposure. If you have risk factors, you should 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) connected by a system of small vessels.

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

Men develop non-Hodgkin's lymphoma much more often than women, and their age at the time of diagnosis varies widely.

The occurrence of non-Hodgkin lymphoma

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

Course of the disease Non-Hodgkin lymphoma

Variants of non-Hodgkin's lymphomas (lymphosarcoma) 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.

Intermediate grade non-Hodgkin's lymphomas:

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

High-grade non-Hodgkin's lymphomas:

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

Separate sections of the WHO classification include mycosis fungoides, reticulosarcomas (according to modern concepts, most of the latter are represented by lymphoid tumors and a small part - by histiocytic variant), plasmacytoma, and unclassified lymphomas.

For nodular lymphosarcoma characterized by the formation of pseudofollicular structures, which, unlike true follicles, are determined both in the cortical and medulla layers of the lymph node, are large in size, have unclear contours and a relatively monomorphic cellular composition.

The diffuse type of growth is characteristic of all variants of non-Hodgkin lymphoma. Diffuse lymphocytic lymphosarcoma characterized by a total proliferation of cells such as small lymphocytes that infiltrate the walls of blood vessels, which leads to 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 characterized by a combined proliferation of lymphoid and plasma cells; plasmatized lymphocytes are also found. Changes in this variant of lymphosarcoma are similar to the picture found in Waldenström's macroglobulinemia; the disease is often combined with various types of monoclonal gammopathies.

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, the 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 practical recovery for most patients in stage I of the process.

In diffuse lymphoblastic lymphosarcoma, proliferations of lymphoblast-type cells are detected, among which there are macro- and microgenerations. Cells with nuclei of a brain-like (twisted, convolutional) shape can be detected. They are more often observed in children when the process is localized in the lymph nodes of the mediastinum and are usually of 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 characterized by massive proliferation of large mono- or multinucleated cells with a large centrally located nucleolus and an abundant zone of basophilic cytoplasm. A large number of mitoses and dying cells are detected. 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 It is distinguished by a monomorphic proliferation of blast cells of the lymphoid type with hyperbasophilic, often vacuolated narrow cytoplasm. Against this background, a typical, although non-specific, feature is the presence of large macrophages, which create a picture of a “travelled palate”. There is an opinion about the proximity of Burkitt lymphoma cells to partially blast-transformed B lymphocytes. Unlike other forms of non-Hodgkin lymphoma, the tumor is primarily localized

extranodal.

At reticulosarcoma(histiocytic lymphoma), a relatively rare tumor, reveals proliferations of cells with morphological and functional signs 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. Some cells exhibit the ability to phagocytose. The cells are characterized by high activity of nonspecific esterase, the ability to secrete lysozyme, and the absence of specific markers.

Non-differentiable 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 also used. Thus, some authors propose to subdivide lymphosarcoma depending on the primary localization of the process; The term “lymphocytoma” emphasizes the benign nature of extramarrow tumors, consisting predominantly of mature forms of small lymphocytes (or lymphocytes and prolymphocytes), forming a structure of nodular growth. Therefore, they are separated from low-grade non-Hodgkin lymphomas into a separate group of lymphoid tumors.

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

Symptoms of Non-Hodgkin's Lymphoma

For all morphological variants of non-Hodgkin's lymphomas, damage to both the lymph nodes as a whole and their individual groups, Waldeyer's lymphoid ring and the gastrointestinal tract is equally common. More frequent primary lesions of the retroperitoneal lymph nodes and abdominal cavity, bones and soft tissues are observed in lymphoblastic, and spleen - in prolymphocytic variants. The pathological process, regardless of the morphological variant of the disease, in most cases first spreads to the areas adjacent to the lymph nodes. Damage to adjacent groups of lymph nodes more often occurs with the lymphoblastic variant.

Early extranodal metastasis, metastasis to the bone marrow, involvement of the liver and spleen in the pathological process are somewhat more common in the prolymphocytic variant, and bone marrow damage and leukemia are more common in the presence of cells with a round and split nucleus. However, with blast variants, bone marrow involvement and an increase in the size of 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 a split and round nucleus is 70 and 53%, respectively. With the prolymphocytic-lymphoblastic variant of large cells with a split nucleus, survival rates are close to those with blast variants and amount to 14-21 months.

Survival rates in stages I-II of non-Hodgkin lymphomas with a high degree of malignancy with primary lesions of the gastrointestinal tract are significantly higher than 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 lymphosarcoma. Somewhat more often, primary damage to the spleen is detected in the prolymphocytic variant. More often, with splenic lymphoma, involvement of the bone marrow in the pathological process is noted. However, with 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 lymphomas. The prognosis for this primary localization is determined by the morphological variant. Damage to the nervous system is observed, as a rule, in blast variants of non-Hodgkin lymphomas.

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

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

Generalization of the process occurs on average in 3-24 months. Bone marrow damage can also be detected with a normal hemogram (in 47% of patients it is unchanged 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 more than 5 years).

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

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

Prolymphocytic variant found in 45-51% of all cases of lymphosarcoma. It often reveals an increase in the occipital, parotid, popliteal, and lymph nodes. Despite the moderate generalization and frequent leukemia (in 25-45%) of the process, with this option the five-year survival rate of patients is 63-70%. With the prolymphocytic-lymphoblastic subvariant, the prognosis is less favorable.

Lymphoblastic variant quite heterogeneous in its morphological (with a twisted, untwisted nucleus, macro-, microforms) and immunological (T- and B-phenotype) characteristics, most often found in children. Lymph nodes of various locations are affected. The disease is characterized by the rapid growth of tumors and the involvement of new anatomical zones in the process. More often than with other lymphosarcoma, the hemogram shows initial cytopenia and a T-cell phenotype of lymphocytes.

Burkitt's lymphoma of B-cell origin is classified as a lymphoblastic type of lymphosarcoma. Its classic version is manifested mainly by damage to the bones (especially the lower jaw), kidneys, ovaries, lymph nodes of the retroperitoneal areas, lungs, and parotid salivary glands. Bone marrow is rarely involved in the process. In localized forms, the prognosis is favorable with long-term remissions up to 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 and lungs; in 50% of cases - leukemia. The disease is more often detected in boys in 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's ring, etc. Cytopenia is often detected, leukemia - in rare cases. The disease progresses quickly, the five-year survival rate of patients is 21-32%, however, removal of a solitary tumor can contribute to long-term remission and even cure. Immunoblastic lymphosarcoma as a secondary process is described in multiple myeloma, Waldenström's macroglobulinemia, and other lymphoproliferative diseases.

Mycosis fungoides is a malignant lymphoid tumor, always initially arising in the upper layers of the dermis, consisting of polymorphic T-helper cells. The first manifestation of the disease may be nonspecific inflammation. The diagnosis is verified by histological and cytochemical examination (lymphoid cells give a positive reaction to acid phosphatase, beta-glucuronidase and acid nonspecific esterase). There is a point of view that the early, chronic phase of the disease may be reactive, while the “lymphoblastic” phase represents true malignant transformation. Sezary syndrome, characterized by the appearance in the hemogram of lymphoid cells with a brain-like nucleus, is considered as the leukemic phase of mycosis fungoides.

The histiocytic variant of malignant non-Hodgkin lymphoma is very rare. Its clinical picture is varied. Metastases can be found in many organs. Leukemia and bone marrow involvement are rare, and cytopenia is common.

The nosological affiliation of the identified new forms remains debatable. Thus, Lennert lymphoma, initially 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, high content of immunoblasts, plasma cells, transitions to lymphosarcoma served as the basis for delimiting this disease from lymphogranulomatosis and distinguishing it under the name “Lennert lymphoma” (malignant lymphoma with a high content of epithelioid histiocytes, lymphoepithelial lymphoma, epithelioid cell lymphoma ). A feature of the clinical manifestations of Lennert lymphoma is the frequent involvement of the palatine tonsils and lymph nodes, the elderly age of patients, the presence of polyclonal gammopathy and a history of allergic skin rashes.

It is proposed to include the non-Hodgkin lymphoma that has also been 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, deposition of PAS-positive amorphous masses in the walls of blood vessels. The number of eosinophils and histiocytes varies, but sometimes the number of the latter is noticeably increased. There may be giant cells and small foci of necrosis. A number of researchers regard the changes described above not as malignant lymphoma, but as reactive, associated with disturbances 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 there is a normal or close to normal number of leukocytes with a predominance or normal content of mature lymphocytes. The platelet level is within normal limits; in some patients, their number may decrease to 1*10 9 /l-1.4*10 9 /l after 7-10 years. More often, only a slight tendency towards a decrease in the level of hemoglobin and the number of red blood cells is detected; reticulocytes fluctuate between 1.5-2%. Bone marrow biopsy reveals isolated proliferates consisting of mature lymphocytes; Histological studies of an enlarged lymph node and other affected organs help verify the diagnosis. Malignancy of lymphocytoma with transformation into lymphosarcoma or chronic lymphocytic leukemia is not obligatory, and if it occurs, it often occurs after many months or years.

Diagnosis of the disease Non-Hodgkin 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. It often takes months from the period when the first signs of the disease appear until a true diagnosis is made.

Primary extranodal lesions can occur in any organ where there is lymphoid tissue. Only 15 cases of isolated liver damage have been described, but metastatic damage is found in more than 50% of patients. 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 histological examination of lymph nodes or other tumor formations; their cytological (imprint, punctate), cytochemical and immunological studies are mandatory. For diagnostic purposes and to assess the extent of the pathological process, bone marrow puncture and biopsy are examined.

Lymphocytomas have a favorable course 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 detected. The tumor substrate consists predominantly of mature lymphocytes (or lymphocytes and prolymphocytes), forming a nodular growth structure. After a long time, transformation of lymphocytes into lymphosarcoma or chronic lymphocytic leukemia is possible.

Treatment of 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, and the presence of other diseases.

The fundamental method of treating 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 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, when bones are involved 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 generalization of the process.

Elderly patients with low-grade non-Hodgkin lymphomas, as well as in the presence of severe concomitant diseases, can be treated with monochemotherapy (chlorbutin, paphencil, cyclophosphamide, etc.). In case of primary damage to the spleen, regardless of the morphological variant of the disease, splenectomy is performed, followed by a course of chemotherapy. In case of isolated damage to the stomach, the combined treatment program includes its resection. For skin lesions, chemotherapy is used, including prospidine and spirobromine.

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

For generalized stages of low-grade non-Hodgkin's lymphomas, the programs TsOP, TsOPP, BATsOP, 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 lymphomas also depend on timely use of hematological resuscitation, immunocorrection and antibacterial therapy.

Since hematopoiesis remains intact in some patients for some time, it is possible to conduct polychemotherapy on an outpatient basis, which, with 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 is carried out. When stable remission is achieved, treatment is stopped.

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

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

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

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

Prevention of non-Hodgkin's lymphoma

To date, there is no known method by which the occurrence of non-Hodgkin's lymphoma could be prevented. Most people with non-Hodgkin lymphoma have not been exposed to known risk factors.

Lymphoma is a type of cancer involving immune system cells 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 treatable disease.

Non-Hodgkin's cancer attacks the body's lymphatic system, which is made up of vessels that carry fluid called lymph to the body. When subtypes of B or T cells begin to multiply uncontrollably, malignant transformation occurs. 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.

Rapidly growing lymphoma - prognosis

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

The indicator depends on 5 factors:

Patient's age.
Stages of lymphoma.
The presence of 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 influence 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 favorable prognosis for recovery and survival. In this regard, there are 4 risk groups:

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

Research developed over the past decade indicates 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.

Over the past five years, treatment methods have changed, and new procedures have been developed for the treatment of malignant tumors, which has a positive effect on prognostic data. Global research in recent years shows that about 95% of people in the very low risk group lived for at least 4 years. While with high hazard rates (low survival rate), 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 prognostic factors:

age under 60 years;
Stage I or II;
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 unfavorable factor): 5-year survival rate becomes 91%, 10-year survival rate becomes 71%.
Intermediate risk (2 unfavorable factors): 5-year survival rate - 78%, 10-year survival rate - 51%.
High risk (3 negative factors): 5-year survival rate - 53%, 10-year survival rate - 36%.

Lymphoma survival

The survival rate directly depends on the stage of the cancer. Also, it should be borne in mind that modern clinical trials (for example, such as stem cell transplantation) can reduce the risk of an unfavorable outcome.

Survival also varies significantly depending on the subtype of Non-Hodgkin's lymphoma. Follicular lymphoma has a better prognosis (96%) than diffuse B-cell lymphoma (65%). characterized by readings that reach 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 indicators are quite high - 82%.
Patients with stage 2 disease have an 88% five-year survival rate.
The five-year prognosis from the initial diagnosis of the disease for patients of the third stage includes 63%.
The fourth stage of cancer is represented by rates of 49% within 5 years of the course of the disease from the first diagnosis.

Five-year survival rates for young men and women are highest and decrease 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 ranges from 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 for men increased by 30%, and for 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 differently and respond to drugs in unpredictable ways. This type of tumor is characterized by rapid development, which is why the prognosis for the life of patients with non-Hodgkin lymphoma is unclear.

Malignant types of lymphatic tumor lesions form in lymphoid tissues. The main sign by which non-Hodgkin lymphoma is determined is the lack of response to treatment methods for Hodgkin lymphoma. A separate characteristic factor is germination into organs that are not part of the lymphatic system. During the 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;
compliance of therapeutic techniques.

The tumor itself has morphological properties, clinical manifestations and prognosis that differ from other malignant neoplasms. It began to be called non-Hodgkin's lymphoma since 1982 in accordance with the accepted classification; this type of tumor 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 statistical data as a whole, 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 occurs from T cells, unlike the rest. Experts have not identified direct causes for the development of non-Hodgkin 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 onset of tumor growth. 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 enlargement of the lymph nodes with increased pain. Mostly such manifestations become noticeable in the areas of the groin, neck, and armpits. The secondary symptoms include:

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

Some types of non-Hodgkin lymphoma have separate symptoms.

Lymphoblastic

Lymphoblastic lymphoma is characterized by the development of lesions in the abdominal cavity. The pathology progresses latently, all symptoms are erased, which is why detection occurs in the later stages and a complete cure cannot be achieved. Active metastasis of lymphoblastic lymphoma affects nearby organs:

liver;
spleen;
ovaries;
kidneys;
spinal cord.

At the terminal stage of the disease, nervous system paralysis occurs, which leads to additional damage to the body. The only method of treatment is to inhibit the development of the tumor and cause the tumor to undergo 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 pain. 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 when bone marrow structures and tissues are affected.

Lymphoma of the intestine

Nyhodgkin's lymphoma of the gastrointestinal tract is often extranodal in type of development, and the process itself is classified as secondary development after metastasis. Symptoms of the pathology include:

pain in the abdominal area;
flatulence;
enlarged spleen;
gagging;
intestinal obstruction;
decreased appetite;
blood in feces.

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 most often occurs in older people and begins in the marginal part of the spleen. At the beginning of the development of the pathology, there are no symptoms, but as it progresses they are expressed as:

increase in temperature in the evening;
sweating during sleep;
rapid weight loss;
rapid saturation during meals;
heaviness in the hypochondrium on the right;
anemia.

Splenic lymphoma is characterized by extranodality, which causes severe pain. Gradually the process spreads to the bone marrow structures. A significant enlargement of the spleen in the later stages of tumor 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 by tests and instrumental studies.
Diagnosis requires the appointment of procedures:

laparoscopy;
lymph node biopsy;
ultrasonography;
thoracoscopy;
lymphoscintigraphy;
tomography (MRI or CT);
bone marrow puncture.

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

Types of lymphosarcoma

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 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, tenderness and swelling of the inguinal, cervical and axillary areas of the lymphatic system.
Diffuse large cell. Specific plaque-shaped rashes and ulcers appear on the skin.
Marginal lymphosarcoma. It is characterized by increased pain in the area of origin, 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 often. Type T lymphomas include:

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

T/NK cell neoplasms are classified as aggressive lymphosarcoma. The disease develops rapidly and its progression is unpredictable, and the possibility of surviving 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 correlate with some parameter. Based on the rate of pathological progression, the following are distinguished:

indolent;
aggressive;
highly aggressive.

According to histological examination, it is determined:

reticulosarcoma;
lymphosarcoma;
T-cell lymphoblastoma;
lymphosarcoma of diffuse type;
primary lymphomas of the nervous system;
follicular lymphomas;
nodular neoplasms;
Burkitt's lymphoma, etc.

The localization parameter of development implies nodal tumors (limited to damage to the lymphatic system) and extranodal (the developing tumor spreads to other organs and systems of the body). Based on cytological parameters, small cell and large cell neoplasms are distinguished.

Treatment, stages and prognosis

Combining different directions of influence on the disease gives the best results. In case of abnormal development, 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 of which a stable remission occurs.

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

After determining the type of pathology identified in the patient, the doctor must determine the extent of the lesion and prescribe a scheme for influencing the tumor. Classic treatment options for non-Hodgkin lymphoma are:

For stage 1-2 lymphoma, it is recommended to prescribe a course of chemotherapy. It is possible to increase the chances of tumor regression by paralleling the course of chemotherapy with a stem cell transplant.
Stage 3-4 NHL requires chemotherapy. The main goal of such treatment is to introduce the disease into a state of long-term remission.
The use of radiation therapy may be prescribed in cases of lesions with the t-cell form of non-Hodgkin lymphoma. The resulting remission can last for several years.
Alternative treatment methods may be used - immunotherapy, hormone therapy or stem cell and bone marrow transplantation.

Alternative methods quite often help to increase 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;
Immune drugs are additionally used.

Radiation therapy is used for isolated tumors or in cases where the use of chemotherapy is impossible or contraindicated.

stages

When considering non-Hodgkin lymphoma from the point of view 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 characterized by low severity, 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 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 is worth noting that in the terminal stage, to determine the form of treatment and the degree of risks, the initial localization of the disease does not matter.

Forecast for life

In the classic situation, when a lymphatic tumor develops, the length of time patients live is calculated using a special scale. Each of the negative signs for the disease is equal to 1 point, and after they are determined, a general calculation occurs:

The prognosis is considered favorable if the NHL is assigned no more than 2 points.
If there are 2 to 3 signs, the prognosis is considered uncertain.
When the scores rise to 3-5, it is generally accepted that the prognosis is negative.

One of the factors that is taken into account in the calculation is the frequency of relapses of the pathology. Most often, favorable prognosis is given to patients with a b-cell type of tumor, but this is rare with a t-cell type.
To roughly predict life with non-Hodgkin's type of lymphoma, there is a statistical table for a five-year period:

Summary statistics to determine 5-year survival risks for 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 when the patient is 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 WHO-ECOG score (from 2 to 4).

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

Low risk category, implying the complete absence of the listed factors. Survival for 5 years or more in 83% of cases.
Low intermediate risk category, implying 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 over a 5-year period averages 46% of cases.
High risk category, implying the presence of three factors from the ECOG list. The average survival rate is 5 years or more in 32% of cases.

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

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

What is the disease

To understand what non-Hodgkin lymphoma is, you need to understand the concept of lymphoma in general. Lymphoma is a name that unites a group of cancer diseases. Cancer cells affect the lymphatic tissue, the lymph nodes greatly increase in size, and 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 lymphoma are not yet fully understood. The following factors can indirectly influence the development of the disease and further prognosis:

working 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 lymphoma somewhat more often than women. Also, the likelihood of detecting the disease increases with age: according to statistics, the majority of those affected are people over 40 years of age. The form of the disease often depends on age: children and young people often experience symptoms of the small cell form of the disease or Burkitt's lymphoma, while older people usually have follicular lymphomas.

Classification of different forms of the disease

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

Nature of the disease

Based on this feature, non-Hodgkin lymphomas are divided into two types: aggressive and indolent lymphomas, which progress rapidly, literally “gushing out” with various symptoms and spreading throughout the body with 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 patient’s death. In addition, indolent type lymphoma tends to degenerate into diffuse large cell lymphoma, which will significantly increase malignancy and, accordingly, worsen the prognosis.

Tumor localization

Although the name "lymphoma" suggests that it primarily affects the lymph nodes, lymphoma may not only be located there. Depending on the location, the following types of lymphomas are distinguished:

Nodal. In this variant, 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 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, so 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 (rolled cells), immunoblastic (a strong proliferation of cells around the central cell is observed - nucleoli) and an undifferentiated type.

Symptoms of the disease

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

Severe enlargement of lymph nodes (usually cervical and axillary).
Weakness, lethargy.
Fever.
Quite fast weight loss.
Sweating heavily even in cool or cold weather.
Headache.

All of the above symptoms are quite common, so it is quite difficult to identify lymphoma in the early stages. However, if you do find an enlarged lymph node, you should immediately consult a specialist, because enlarged lymph nodes can occur either from a common cold or from such a serious disease as lymphoma.

How is diagnostics carried out?

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

Histological examination (puncture or biopsy of the lymph node). This test can detect abnormal cells in the lymph that indicate the presence of lymphoma.
Cytological and cytochemical analyzes based on punctate.
Immunological studies.

When it is possible to find out whether a person has lymphoma, other tests are prescribed that will help establish all the parameters of the disease, because further treatment depends on this.

Stages of disease development

When diagnosing a disease, its malignancy and stage of the disease are determined. The malignancy may be high, low or intermediate. As for the stages, there are four of them.

Stage I

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.

Stage II

At the second stage of a disease such as non-Hodgkin lymphoma, the tumors become multiple. General symptoms gradually begin to appear. If we are talking about the B-cell form, then at this stage the neoplasms begin to be divided into those that are subject to and those that are not subject to removal.

Stage III

At this stage of 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, unfortunately, is very disappointing. The disease affects not only organs, but also the bone marrow, reaching the central nervous system and bones. If the treatment was delayed so much that the lymphoma entered this stage, then there can be no talk of recovery.

What treatment methods are used in the fight against 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, stage and malignancy. But in general, the following ways to combat the disease can be distinguished:

Chemotherapy. It is this that 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 radiation in its pure form can only help at stage I. Radiation therapy is also sometimes used locally in those areas where the tumor behaves most aggressively.
Surgical removal of the tumor. Of course, this method is used only if the stage and type of tumor allows. With successful use of radiation therapy and surgical intervention, the prognosis is quite positive: remission may occur for a period of 5 to 10 years.
Application of programs BATSOP, TsOP, etc. I must say, these programs show quite good indicators.
Palliative treatment 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 poor and the chances of defeating the disease are very low. In addition to medications, the patient may need the help of a psychologist, as well as the support of relatives.

What is the prognosis for the outcome of the disease?

As already mentioned, treatment, as well as the effect of it for non-Hodgkin lymphoma, is a very individual matter. Some people manage to achieve a complete recovery on the first try, others have to face relapses, others can only extend their life by a few years, and in some cases, medicine is completely powerless. However, the diagnosis should not be considered a death sentence: with timely diagnosis, proper treatment and a not too problematic form of the disease, the chances of recovery reach 65–70%. And this is quite a lot.

Is everything in the article correct from a medical point of view?

Answer only if you have proven medical knowledge

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