Non-Hodgkin's lymphoma - what is it and how to treat it? Non-Hodgkin's lymphoma Non-Hodgkin's malignant cellular lymphoma

Non-Hodgkin's lymphoma (NHL) is a whole group of cancer diseases, uniting 30 pathologies. They are characterized by the formation of cancerous tumors that affect the human lymphatic system.

With non-Hodgkin's lymphoma, the survival prognosis is very low: about 25%. The peculiarity of this pathology is that it is not affected by traditional treatment methods.

About the disease

What is non-Hodgkin's lymphoma? It is one of the types of lymphatic tumors. The cells of the neoplasm differ in structure from Hodgkin lymphoma, and therefore the methods of treating the latter pathology are not suitable for the treatment of the disease in question. In addition, the further development of NLZ is difficult to predict.

In many cases, non-Hodgkin lymphomas grow into organs that have nothing to do with the lymphatic system. The pathology also affects lymphatic tissues, which are found in:

  • thymus, or thymus gland;
  • spleen;
  • tonsils;
  • small intestine.

The disease mainly affects older people due to the characteristics and causes of its development. In children, NHL develops very rapidly, provoking the appearance of other severe pathologies, which ultimately leads to death.

According to official medical statistics, non-Hodgkin's lymphoma is diagnosed in approximately 4% of people diagnosed with cancer.

Causes

The reasons that provoke the development of the pathology in question have not yet been fully studied. Most non-Hodgkin's lymphomas are formed from so-called B cells, but a number of tumors are formed due to mutation of T cells. It is known that even after the occurrence of the latter process, not all children subsequently develop a malignant tumor in the lymphatic system.

What is known about the reasons for the development of this pathology is that it appears due to the influence of a number of factors:

  1. Infection of the body with viruses of various etiologies (HIV and others).
  2. Congenital pathologies of immunity.
  3. Stomach ulcer, the long course of which provoked the appearance of Helicobacter pylori. In this case, lymphoma occurs in the gastric tissues.
  4. Age-related changes in the body.
  5. Organ transplantation in which the human immune system is suppressed.
  6. Obesity.
  7. Chemotherapy and radiation used in the treatment of other cancer diseases.

The environment has a significant influence on the state of the body. Negative environmental conditions and regular contact with chemicals contribute to malignant cell mutation. In some cases, non-Hodgkin's lymphoma develops due to long-term use of medications.

Symptoms of the disease

The manifestation of symptoms of non-Hodgkin lymphoma depends on the nature of the disease itself. If the pathology develops aggressively and is characterized by increased malignancy (more often observed in children over 5 years of age than in older people), then the presence of a neoplasm is indicated by swelling of a small area of ​​skin resulting from an increase in the size of the lymph nodes.

They are mainly localized:

  • on the head and back of the head;
  • in the groin area;
  • in the armpits.

The development of such neoplasms in most cases is not accompanied by the appearance of pain. Although the tumor appears in these areas, it can develop in other parts of the body: the chest or abdominal area.

Gradually, pathogenic cells spread throughout the body, affecting the central nervous system, liver and spleen. This leads to the formation of symptoms characteristic of damage to these organs.

The presence of non-Hodgkin's lymphoma is indicated by an increase in body temperature. Later, the patient experiences a sharp decrease in weight, which can occur against the background of an increase in appetite. Malignant lymphoma also manifests itself in the form of:

  • profuse sweating at night;
  • general weakness;
  • increased fatigue;
  • itching felt over the entire surface of the body;
  • fever.

Among the obvious signs that may indicate the formation of a malignant tumor in the lymphatic system are symptoms of anemia. They are characterized by increasing cases of infection of the body against the background of a decrease in the number of white blood cells. The location of the neoplasm is indicated by the following manifestations:

  1. Pain in the abdominal area. Intestinal disorders, expressed in the form of vomiting, diarrhea and constipation. These signs indicate that the lymphoma has formed in the abdominal cavity.
  2. Chronic cough and shortness of breath. They report damage to the lymphatic tissue in the chest.
  3. Joint pain.
  4. Headache, blurred vision and vomiting. These symptoms indicate damage to the brain and other central nervous system organs.

Another specific sign of malignant lymphoma is localized skin bleeding, or petechiae. They occur due to a decrease in platelet levels.

The signs described above are characteristic of other types of diseases, including infectious pathologies. However, in cases where the symptoms of the disease sharply intensify their manifestation within 2-3 weeks, then we are probably talking about non-Hodgkin's lymphoma.

Stages of development of the disease

Like other forms of cancer, non-Hodgkin's lymphomas develop through several stages:

  1. First stage.
    The first stage of the pathology is characterized by local damage to the lymphatic system, excluding the sternum and abdominal region.
  2. Second stage.
    The affected area spreads to other parts of the lymphatic system. Cancer cells penetrate the gastrointestinal tract.
  3. Third stage.
    In the third stage, lymphoma is found on both sides of the diaphragm. Subsequently, the tumor penetrates into the abdominal cavity.
  4. Fourth, or terminal stage.
    It is characterized by a severe course and a negative development prognosis. At this stage, cancer cells have spread throughout the body and affected the central nervous system organs, including the bone marrow. In addition, metastases are observed in the bones.

Lymphoma classification

The type of lymphoma in question is classified according to several criteria. Depending on the rate of tumor development, such neoplasms are divided into:

  • Indolent. The pathology proceeds slowly and has a favorable prognosis.
  • Aggressive.
  • Highly aggressive. In most cases they lead to death.

Depending on the location, cancerous tumors are divided into:

  1. Nodal. The tumor affects only the lymph nodes.
  2. Extranodal. Non-Hodgkin's lymphoma occurs in organs that do not belong to the lymphatic system.

Above were the main classifications accepted in medical practice. According to their structure, lymphomas can be either small-cell or large-cell. There are also other types of tumors, characterized by their own characteristics.

Examples include chronic lymphocytic and B-cell prolymphocytic leukemia, plasma cell myeloma, and extraosseous plasmacytoma. It was stated above that in medicine it is customary to distinguish about 30 types of non-Hodgkin lymphoma.

Some of them will be discussed in more detail below.

Lymphoblastic tumor

This type of tumor is characterized by damage to the abdominal region. In most cases, the disease develops asymptomatically. The presence of a tumor becomes known in the last stages of development or during examination of the specified area. Due to the described cause, approximately 90% of patients with lymphoblastic tumor die.

One of the main signs of this form of cancer is active metastasis. Pathogenic cells affect the liver, spleen, spinal cord and other organs. The fourth stage of tumor development is accompanied by paralysis of the nervous system, which subsequently leads to irreversible and fatal consequences.

Treatment of lymphoblastic tumor involves the use of methods that inhibit the development of the tumor. Surgical intervention in these cases is ineffective due to the fact that metastases spread very quickly throughout the body.

Lymphocytic tumor

Like the previous type of tumor, lymphocytic tumor develops asymptomatically for a long time. Its presence may be indicated by tumors on the surface of the skin that appear as a result of the proliferation of lymph nodes. This neoplasm develops from mutated B cells. The pathology is mainly encountered by middle-aged and elderly people.

Due to its asymptomatic course, a lymphocytic tumor is usually detected at the moment when it has reached the fourth stage of development, and metastases have affected the bone structure.

One of the main signs indicating the presence of a malignant formation in the body is enlarged internal organs. In the initial stages, cases of damage to the respiratory system and spleen are common.

Tumor in the intestine

Intestinal lymphoma occurs quite often among patients faced with this pathology. However, such a tumor mainly develops as a result of metastasis of a neoplasm that has arisen in other organs. Among the characteristic signs indicating lymphoma in the intestine are the following:

  • dyspepsia;
  • pain in the abdominal area;
  • spleen growth;
  • flatulence;
  • constipation;
  • nausea;
  • the appearance of blood clots in the stool;
  • decreased appetite.

As a rule, this form of lymphoma develops due to a mutation of B cells that occurs against the background of immunodeficiency.

Tumor in the spleen

Damage to the spleen is observed mainly in elderly people. During the initial stages of development, the tumor does not manifest itself in any way, but later the following symptoms indicate its presence:

  • sudden weight loss;
  • a feeling of heaviness arising in the right hypochondrium;
  • increased sweating during sleep;
  • anemia;
  • tumor growth leads to rapid satiety, even if the person has eaten little food;
    hyperthermia.

Pain occurs after the tumor begins to grow into neighboring tissues. Subsequently, cancer cells are detected in the blood and bones.

Diagnostics

Diagnosis of lymphoma aims not only to establish the presence of a tumor, but also to identify its current form. For these purposes, many different examinations are carried out, including blood tests, ultrasound, and x-rays. A biopsy of the affected organ is required.

If a tumor is detected in a lymph node, the latter is removed.

Additionally, fluid is taken from the abdominal cavity if a tumor is detected in this area. To examine the bone marrow, a puncture is performed. After the form of the disease has been established (based on flow cytometry, immunological and other types of tests), an ultrasound is prescribed, which makes it possible to determine the extent of the prevalence of cancer cells in the body.

In addition to these procedures, MRI, CT and positron emission tomography (PET) are performed. At the same time, ECGs are performed to detect obvious signs of the influence of a malignant tumor on the heart and metabolic processes. The latter method also allows you to determine the presence of infections in the body.

Treatment and prognosis

Treatment of non-Hodgkin lymphoma involves a set of measures aimed at suppressing the active development of the tumor and its removal. To achieve these goals, radiation, chemotherapy and surgery are used.

Surgical removal of the tumor is resorted to only when the lymphoma is located in a limited area and has not metastasized. Radiation therapy is also used when the disease is local. The latter method is prescribed provided that chemotherapy has not yielded positive results.

To treat lymphomas, a method known as polychemotherapy is used in most cases. It allows you to increase the patient’s life expectancy and suppress the main symptoms.

An alternative to the described methods is the use of a combination of several drugs that have an immunotherapeutic effect. Stem cells, bone marrow transplantation, or the use of monoclonal antibodies can also stop the development or completely destroy a malignant neoplasm.

To predict the development of the disease, a special scale is used today, according to which:

  1. 0-2 points indicate a favorable outcome of treatment. This usually applies to tumors that arise from B cells;
  2. 2-3 points. The prognosis is uncertain;
  3. 3-5 points. The survival rate among patients tends to zero, and therapy is aimed at extending life expectancy.

Points are awarded for each unfavorable sign identified during the examination of the patient.
If the above symptoms appear, it is not recommended to conduct self-diagnosis and prescribe treatment based on the data obtained.

To increase your chances of survival and successful recovery, you should see a doctor. The oncologist better understands the dangers of non-Hodgkin lymphomas, what they are, and how to cope with the pathology.

– 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's lymphomas varies, depending mainly on the histological type of 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 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.

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:

  1. Large cell. They are characterized by a clear increase in the axillary and cervical nodes of the lymphatic system.
  2. Follicular. There is persistent redness, tenderness and swelling of the inguinal, cervical and axillary areas of the lymphatic system.
  3. Diffuse large cell. Specific plaque-shaped rashes and ulcers appear on the skin.
  4. 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:

  1. The prognosis is considered favorable if the NHL is assigned no more than 2 points.
  2. If there are 2 to 3 signs, the prognosis is considered uncertain.
  3. 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
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.

Valery Zolotov

Reading time: 8 minutes

A A

It is an oncological disease. With it, the lymph nodes enlarge and various internal organs are affected, in which white blood cells (lymphocytes) accumulate.

Such master cells in the immune system are transmuted and become malignant. These cells begin to divide, without control, and gain multinucleate status. As a result, they begin to destroy everything around them.

Lymphoma is classified by size and degree of its development in the human body. When making a diagnosis, it is necessary to know this important parameter. It is impossible to determine the full picture of the disease if the degree of destruction of the body is not established.

Doctors determine the stages of lymphoma in the following ways:

  1. conduct microscopic examinations;
  2. take tests;
  3. visualized and determined.

Their job is to accumulate and cause diseases along with malignant neoplasms, while:

  • the entire skeletal system begins to ache;
  • pain is felt in the left hypochondrium because the spleen is enlarged;
  • It becomes difficult in the right hypochondrium, since the liver area is affected. Symptoms appear in the form of jaundice, heartburn;
  • dull aching pain covers the abdominal area. In addition, shortness of breath appears;
  • dropsy occurs;
  • fluid accumulates in the liver;
  • the lymph nodes begin to ache due to the fact that the growth of malignant cells does not stop;
  • breathing is difficult, so the person begins to cough. Symptoms that the lungs and pleura are affected. The patient may cough up blood;
  • the chest begins to hurt very much;
  • the disease can cause paralysis. The legs may go numb as metastases occupy the spinal cord;
  • a bluish color appears on bumps protruding on the body. They are called compactions - infiltrates.

Diagnostics

In order to confirm the disease, three main diagnostic methods are used.

Using laboratory diagnostics, the patient is sent to:

During instrumental diagnostic examination:

  1. the patient is sent for examination using x-rays and chest ultrasound to determine therapeutic volumes;
  2. the patient is sent for a computed tomography scan (to visualize enlarged lymph nodes and assess their further condition);
  3. MRI scans the lungs to detect bone malignancy and disease in the central nervous system.

For radiological diagnosis:

  1. the patient undergoes lymphoscintigraphy tests to determine the condition of the lymphatic vessels and limbs. Lymphoscintigraphy provides information through images that show the lymphatic vessels in the legs, how the peripheral lymph flow passes, and determines pathologies associated with lymph in the vessels and nodes;
  2. determine the degree of spread of cancer cells using positron emission tomography. A substance that has accumulated in the affected organs is injected into the blood;
  3. The diagnosis of hydrops is determined by imaging if there is a large amount of fluid in the abdominal cavity. If there is little fluid in this area, then percussion of the abdomen (percussion) is used. This can be detected by sending the patient for an ultrasound or CT scan.

Treatment of stage 4 lymphoma

Here the disease manifests itself in its advanced form. Therefore, in this case there is no good prognosis. There is only a low chance of recovery. But she is!

When such a disease is confirmed, treatment with combined chemotherapy and radiotherapy is started immediately by spleectomy (removal of the spleen). This achieves partial remission (weakening of lymphoma), slowing down the spread of the disease.

Chemotherapy treatment is prescribed. For a more effective method, a combination is used that includes cytostatic drugs and. In this case, the drugs are taken according to highly effective regimens.

Radiotherapy is used on affected areas when there is no certainty that there are no cancer cells left after chemotherapy sessions.

New treatments include radioimmunotherapy. With this treatment, antibodies that are tagged with radiation are injected. Such bodies have the ability to stick to diseased cells, destroying them.

If chemotherapy sessions are carried out at high doses, causing the risk of destroying healthy cells, then bone marrow or stem cell transplantation operations are performed. After courses of chemotherapy, they can be returned to their places thanks to the veins.

Transplant operations can be performed using donor cells. Hydrops or ascites are treated at this stage.

Paracentesis (puncture) is performed to drain the fluid and install a catheter. Radioisotopes, biological agents, and chemotherapy are injected into the abdominal cavity.

Adjuvant treatment with diuretics is prescribed.

When treating such a serious illness, they first of all focus on the psychology of the patient. After all, if he fights for his life and does not give up, the body will begin to resist diseases. And then the effectiveness of therapeutic treatment will increase significantly. Most deaths occur due to intoxication, when secondary infections are barricaded in the body, and anemia.

Stage 4 Predictions

Although the prognosis in this case is not good. And how many are left, no one knows, you still have to hope and fight, because 60% of 100 have a five-year survival rate. If modern intensive methods are used, then they should not be abandoned. With this treatment, the prognosis increases to 90% in terms of survival. In this case there is no difference from early stage survival.

The main thing is how the patient will feel. This is a determining factor in forecasts.


How long do people live with a brain tumor?
(Read in 4 minutes)
Lymphoma disease: signs, symptoms and treatment
(Read in 7 minutes)

This group covers the entire spectrum; from B-cell neoplasms of low malignancy, which are incurable, but can last for years, to lymphomas with a high degree of malignancy, without treatment, quickly leading to death, but when treated with modern methods, ending in recovery for a fairly significant number of patients.

What is non-Hodgkin's lymphoma

Non-Hodgkin's lymphoma is the second most common malignant tumor affecting patients with AIDS.

Non-Hodgkin's lymphoma of bone is a rare tumor, accounting for 1% of all non-Hodgkin's lymphomas, and often generalizes within a few months of discovery. In half of the cases, primary bone lymphosarcoma develops in people over 50 years of age.

Causes of non-Hodgkin's lymphoma

The risk of developing lymphoma in people infected with HIV is 60-160 times greater than in people who do not have this infection. The incidence of NHL increases as immunosuppression worsens. Almost half of the patients have a history of AIDS-defining diseases. Non-Hodgkin's lymphomas are also more common in people with other immunodeficiencies.

There is a close relationship between the development of NHL in HIV-infected people and EBV carriage. EBV proteins can be detected in more than 50% of patients with lymphoma, especially immunoblastic and large cell lymphoma. It is assumed that infection of EBV cells in an HIV-infected patient contributes to their uncontrolled proliferation. Oncogenic mutations have also been identified, for example in the p53 and C-tue genes.

The incidence of NHL is increasing. In the United States, it has increased by 3-4% annually since the 1970s and currently stands at approximately 15 cases per 100,000 population. The pathogenesis of most NHL remains unclear, but some etiological factors have been identified.

  • Longevity.
  • Long-term immunosuppression, for example congenital immunodeficiencies, HIV infection (AIDS-associated HXL), post-transplant lymphoproliferative disease.
  • EBV infection in Burkitt's lymphoma, lymphoma in HIV-infected patients and patients with organ transplants.
  • Helicobacter pylori infection in intestinal lymphomas.
  • Chlamydial infection in lymphomas from marginal zone cells of the ocular appendages.
  • HCV in marginal zone cell lymphomas.
  • Systematic hair coloring.

Approximately 80% of cases of NHL in HIV-infected people are highly malignant.

In 90% of cases or more, the tumor is represented by immunoblastic or berlitt-like lymphoma.

In patients with immunodeficiency, lymphoma is not always monoclonal. Polyclonal lymphomas are sometimes encountered, which indicates their high tendency to metastasize.

Non-Hodgkin's lymphoma (lymphosarcoma of bone) was previously considered to be reticulosarcoma of bone. Later, based on clinical, histological, immunophenotypic and genetic data, it was established that tumor cells of bone reticulosarcoma and extraskeletal malignant lymphomas have a single lymphoid origin. In fact, bone reticulosarcoma is an analogue of lymphoma that occurs extraskeletal, most often in the lymph nodes. In contrast to lymphogranulomatosis, this tumor began to be designated as non-Hodgkin's lymphoma or primary lymphosarcoma of bone.

Typical locations are vertebrae, flat bones (pelvis, ribs, sternum) and metaphyses of long tubular bones. Often the disease begins with a pathological fracture.

Morphologically, all bone lymphomas are divided into low-, moderate- and highly differentiated forms. Most lymphosarcoma of bone are large cell or mixed small-large cell diffuse forms (often B-cell, rarely T-cell and KT-cell). Lymphosarcoma cells are characterized by more pronounced polymorphism compared to tumors of the Ewing sarcoma family and other small round cell sarcomas and are surrounded by a distinct network of reticulin argyrophilic fibers. The presence of reactive lymphocytes sometimes makes diagnosis difficult.

Classification of non-Hodgkin's lymphomas

Immunological identification of lymphocytes and molecular analysis of immunoglobulin and gene rearrangement of its receptor in T lymphocytes made it possible to develop a more advanced classification of NHL based on biological rather than morphological characteristics of cells. Most non-Hodgkin's lymphomas are B-cell.

The WHO classification is currently used. It is based on the pathology of NHL, their origin (T- or B-lymphocytes) and the collective opinion of experienced specialists about whether a given tumor belongs to one or another type of lymphoma.

In everyday practice, the clinical features of lymphoma, in particular its malignant potential, are the most important parameter when choosing treatment tactics. They are taken into account in a classification that divides these tumors into two large groups: lymphomas of low (“sluggish”, low-grade) and lymphomas of high (“aggressive”, nigh-gracle) degree of malignancy.

Symptoms, signs and stages of non-Hodgkin's lymphoma

The tumor manifests itself clinically in the advanced stage of HIV infection.

Lesions of extranodal localization are often detected. In 80% of patients, the tumor process at the time of diagnosis corresponds to stage IV and occurs with the involvement of the gastrointestinal tract, bone marrow, central nervous system, liver, as well as recurrent cancerous effusion in the body cavities.

Differential diagnosis includes tuberculosis and cytomegalovirus infection.

Most adult patients (60-70%) with NHL consult a doctor with complaints of enlarged lymph nodes, while in children the reason for referral is usually extranodal lesions. Typically, there is painless enlargement of the lymph nodes in one or more anatomical areas. In low-grade NHL, lymph node enlargement may persist for a long time or progress slowly, while in high-grade lymphoma, the lymph nodes continue to enlarge rapidly. Hepatosplenomegaly is often noted. Extranodal lesions are characterized by a variety of clinical manifestations. These lesions affect the intestines, testicles, thyroid gland, bones, muscles, and lungs. Central nervous system, paranasal sinuses, skin. Common symptoms include night sweats, weight loss, and fever with negative blood cultures.

To clarify the diagnosis, a biopsy of the lymph nodes is performed; if there is no lymphadenopathy, then a biopsy of the extranodal lesion. The resulting material is subjected to immunohistochemical, cytogenetic, molecular and morphological studies.

The stage of the tumor process is determined not on the basis of pathomorphological (including surgical intervention) assessment, but according to the results of a clinical and laboratory examination:

  • chest x-ray;
  • clinical blood test and blood smear examination to detect leukemia of the tumor;
  • aspiration biopsy and bone marrow trephine biopsy for morphological and cytogenetic studies, as well as immunophenotyping;
  • determination of biochemical indicators of liver and kidney functions, calcium and uric acid content in blood serum;
  • determination of markers of the tumor process - LDH activity and the amount of p-2-microglobulin;
  • other studies - depending on the clinical characteristics of the disease (CT of the head, MRI of the spine, lumbar puncture, bone scintigraphy).

The clinical classification of NHL stages is a modification of the Ann Arbor classification of stages of lymphogranulomatosis.

X-ray and CT scans reveal bone destruction in the form of “eatenness”, “sponginess”, similar to “melting sugar”. Sometimes merging osteolytic foci have unclear contours and create a picture of local rarefaction of the bone structure, which, in the absence of changes in the cortical layer, periostosis and extraosseous component, can be missed during x-ray examination. MRI is generally recognized as the most sensitive method for determining bone marrow infiltration. As reactive changes increase, the bone acquires a “motley” appearance due to a mixed pattern of small lytic foci with sclerotic contours and areas of reactive bone (Fig. 4.47).

When reactive changes prevail, the bone affected by lymphosarcoma becomes dense. Less commonly detected is lymphosarcoma, which causes a cellular restructuring of the bone structure with disintegration and fragmentation of the cortical layer. As the process progresses, the cortical layer is destroyed in a limited area, mild periostosis and a small extraosseous component appear. If the paraarticular parts of the bones are affected, reactive synovitis may occur.

Diagnosis and stages of the disease

Clinical and biochemical blood tests, including determination of LDH activity.

Chest X-ray. CT scan of the head, abdomen and pelvis.

Biopsy of lymph nodes (preferably with a fine needle) and bone marrow.

Lumbar puncture and cerebrospinal fluid examination, even if there are no symptoms.

At the stage of radiological diagnosis, primary lymphosarcoma of the bone must be differentiated from osteomyelitis, osteosarcoma, chondrosarcoma, Ewing's sarcoma, the rare cellular form of this tumor - from cysts, FD and GCT. When generalizing the process throughout the skeletal system, lymphosarcoma must be differentiated from metastases of breast cancer, small cell lung cancer and myeloma. Each of the listed nosological forms has its own clinical and imaging differential diagnostic criteria, which in most cases allows us to clarify the nature of the damage to the skeletal system. However, sometimes a correct interpretation of the pathological process is possible only after a biopsy of the affected bones.

At the stage of histological examination, differential diagnosis is carried out with other hemoblastoses: myeloid leukemia, anaplastic plasmacytoma, LCH, metastases of small cell cancer and round cell sarcomas: Ewing's sarcoma, rhabdomyosarcoma.

Poor prognostic factors

  • A history of AIDS-defining diseases or a CD4 lymphocyte count of less than 100 cells per liter.
  • Karnofsky score less than 70%.
  • Age over 35 years.
  • Extranodal involvement, including bone marrow infiltration.
  • Increased LDH activity.
  • Immunoblastic subtype of lymphoma.

Treatment of non-Hodgkin's lymphoma

Ideally, the treatment coordinator at the center where the patient is hospitalized should be a specialist with experience in the treatment of AIDS-associated lymphomas. Administration of HAART concomitantly with anticancer therapy reduces the incidence of opportunistic infections and may improve survival. Local treatment may be effective for stage I or II lymphoma, but the vast majority of patients with AIDS-associated lymphoma have stage IV disease, when systemic treatment is necessary.

Treatment for lymphoma consists of combination chemotherapy. The most commonly used regimen is CHOP in 3-week cycles, although other regimens can be used. Excessive intensification of chemotherapy is difficult for patients to tolerate, partly due to immunodeficiency and decreased functional bone marrow reserve. If the meninges are damaged, chemotherapy drugs (methotrexate and cytarabine) are administered subarachnoidally. Prophylactic subarachnoid administration of chemotherapy is indicated if there is a high risk of meningeal involvement (for example, in patients with paraspinal and paranasal Burkitt lymphoma or lymphoma infiltration of the bone marrow) or with a positive cerebrospinal fluid test for EBV.

The effectiveness of treatment (response rate and duration of remission) in HIV-infected patients is lower than in patients with lymphoma of a similar histological structure who do not suffer from this infection. Median survival does not exceed 12 months. Death occurs from relapse of lymphoma or from opportunistic infections. The survival rate of patients who experience longer remission after chemotherapy is 6-20 months, with a small proportion of patients living longer.

Rituximab, a monoclonal antibody, is used to treat NHL in people who do not have HIV infection. However, opinions on the role of NHL in HIV-infected people are controversial. There are no data on its effectiveness, possible reduction in CD4 count and increase in viral load, confirmed in phase III clinical trials.

Low-grade non-Hodgkin's lymphomas

Low-grade NHL accounts for 20-45% of all NHL. They are prone to dissemination, and by the time they see a doctor, patients usually have widespread lymphadenopathy, hepatosplenomegaly, and often changes in the blood and bone marrow picture.

Follicular lymphoma

Follicular lymphoma usually develops in older people, although it can occur in younger people. Occasionally, at the time of diagnosis, the tumor process corresponds to stage I, when radiation therapy is possible. But more often, follicular lymphoma is diagnosed at stage III or IV, when it is incurable. The disease has an undulating course, and the median survival of patients is 6-10 years. Follicular lymphoma can transform into high-grade NHL.

Lymphoma cells are characterized by reciprocal chromosomal translocation, as a result of which the Bcl-2 oncogene from chromosome 18 moves to chromosome 14 and falls into the zone of action of genes that regulate the synthesis of heavy chains of immunoglobulins (IdH). Overexpression of the protein product Bcl-2 prevents the onset of apoptosis (programmed death) of the lymphoma cell, so that the basis of lymphoma from the cells of the center of the follicle is essentially the uncontrolled accumulation of tumor cells.

Principles of treatment

In a small proportion of patients whose disease corresponds to stage I, they resort to irradiation of the affected group of lymphatic angles. Sometimes this leads to recovery, but many patients have latent tumor foci, which subsequently lead to generalization of the tumor process or relapse.

At an advanced stage of the disease (II and more common stages), chemotherapy is prescribed only if the clinical picture is severe.

With advanced but asymptomatic cancer, patients can be enrolled in a UK clinical trial comparing watchful waiting with the effectiveness of rituximab in the hope of delaying chemotherapy.

Patients with clinical manifestations are traditionally treated with first-line chemotherapy with chlorambucil or a CVP or CHOP regimen. Randomized trials conducted in recent years have shown that in terms of response rate and duration of remission, first-line chemotherapy with the R-CVP regimen is superior to therapy with the CVP regimen, R-CHOP is superior to CHOP, and R-MCP (response rate 92.4%, disease-free survival 82 .2%) is superior to MSR (mitoxantrone, chlorambucil, prednisolone) (response rate 75%, disease-free survival 50.7%). The long natural history of follicular lymphoma and the wide choice of chemotherapy regimens for subsequent treatment of patients make it difficult to assess the impact of these regimens on overall survival. However, 30-month survival after R-MCP chemotherapy was significantly higher than after MCP chemotherapy (89.3% and 75.5%, respectively). It is possible that longer duration of rituximab maintenance therapy will further improve treatment outcomes. Currently, it is customary to include rituximab in all of the above first-line chemotherapy regimens.

Treatment when signs of tumor progression appear after the first remission includes drugs such as:

  • purine analogues: fludarabine and 2-CDA;
  • auto- or allogeneic stem cell transplantation;
  • labeled anti-CO20 antibodies;
  • IFN alpha;
  • rituximab (as a palliative measure without other drugs);
  • antisense oligonucleotides to interrupt the synthesis of the Bc1-2 protein.

What should be the optimal combination of numerous treatment methods is currently unknown. Since none of the existing methods cures effectively. Patients with lymphoma usually have time to try all available methods over the years of treatment. The presence of antibodies with auto- or allo-transplantation of stem cells after pre-transplantation chemotherapy in a booster mode is of interest. Once the result of treatment (presence of mild minimal residual disease or its absence) correlates with the duration of the disease, then such intensive therapy may be effective, i.e. cause long-term remission.

Forecast

The prognostic indicator of follicular lymphoma allows us to distinguish three risk categories based on clinical parameters - age, stage of infection, hemoglobin content, LDH activity and the number of affected groups of lymph nodes. The 10-year survival rates in these three prognostic groups were 76, 52 and 24%.

Gene expression profiling also has prognostic implications. Notably, the gene expression patterns of T lymphocytes and monocytes in infiltrating lymph nodes predict survival.

High-grade non-Hodgkin's lymphomas

Tumors of this group include lymphomas that have a pronounced ability to involve the central nervous system - lymphoblastic, Burkitt's lymphoma, adult T-cell leukemia/lymphoma, primary central nervous system lymphomas and lymphomas with a less pronounced tendency to metastasize to the central nervous system. However, the frequency of metastasis to the central nervous system of lymphomas of the latter group increases significantly with multiple extranodal lesions, lesions of the testicles, colonic sinuses, or paraspinal tissues. Such patients need to undergo clinical and instrumental neurological examination and preventive therapy.

Burkitt's lymphoma

Endemic.

  • Burkitt's lymphoma is endemic in the countries of equatorial Africa.
  • 90% of cases of the disease are associated with EBV infection.
  • In children and young people, the disease is clinically manifested by enlarged cervical lymph nodes.

Non-endemic.

  • The role of EBV infection is traced in approximately 20% of cases.
  • Abdominal lesions are more common
  • Associated with HIV infection

Treatment

Short-term intensive therapy with the introduction of methotrexate, cyclophosphamide and ifosfamide into the subarachnoid space. Long-term remission in 50-70% of patients can be achieved, for example, with CODOX-M chemotherapy for low-grade lymphoma or alternating CODOX-M and IVAC chemotherapy for high-grade lymphoma.

Lymphoblastic lymphoma

Lymphoblastic lymphoma manifests itself with leukemia, symptoms of compression of the mediastinal organs by enlarged lymphoid tissue, as well as pleural effusion, is more common in children and usually consists of T lymphocytes. Treatment consists of urgent removal of tumor compression and prevention of tumor collapse syndrome. Intensive chemotherapy according to regimens used for acute lymphocytic leukemia, in combination with treatment of metastases in the central nervous system, has improved the prognosis in children, but in adults the results are still unsatisfactory.

Unfavorable prognostic factors include bone marrow involvement, increased LDH activity more than 300 IU/L, age over 30 years, and delayed response to therapy. Autologous or allogeneic stem cell transplantation may improve survival in patients with poor prognosis.

Diffuse large B cell lymphoma

This tumor, the most common of high-grade NHLs, presents with lymph node involvement or extranodal involvement. Radiation therapy or the more commonly used short-term chemotherapy according to the CHOP regimen, followed by irradiation of the affected group of lymph nodes, can achieve stable remission in 90% of patients in stage IA. In more advanced stages of the disease, chemotherapy according to the R-CHOP regimen is adopted as standard. The feasibility of this therapy was shown in the GELA study, in which the 3-year disease-free and overall survival of patients over 60 years of age who received 8 courses of R-CHOP was 53% and 62%, and for patients who received only CHOP, it was 35% and 51%. In the UK, the National Institute for Care Excellence (NICE) has approved the use of R-CHOP as first-line chemotherapy for diffuse large B-cell lymphoma. In Germany, when treated with the CHOP regimen, supported by the administration of GCSF every 14 days, a higher rate of achieving complete remission was noted (77% and 63.2%, respectively) and a longer period until tumor progression resumed with the administration of GCSF every 21 days compared with CHOP circuit in its purest form.

Recurrent diffuse large B-cell lymphoma

Further treatment of patients with relapses of diffuse large B-cell lymphoma includes high-dose chemotherapy followed by autologous stem cell transplantation. This tactic is accepted as the standard if there are no contraindications to it. However, the results of the meta-analysis did not reveal the advantages of high-dose chemotherapy with autotransplantation of stem cells in the first remission of lymphoma.

Prognosis for diffuse B-cell lymphoma

In accordance with the international prognostic indicator, prognostic groups are distinguished taking into account age, stage of the disease, the number of affected groups of lymph nodes, LDH activity and functional activity. An indicator corresponding to 0 or 1 point corresponds to a low risk group, 2 points - moderate risk, 3 points - moderately high risk and 4-5 points - high risk. 5-year survival with conventional therapy ranges from over 70% for the low-risk group to 20% for the high-risk group.

Recent studies of gene expression in lymphoma cells have identified variants of diffuse B-cell lymphoma with different biological activities, which has significantly improved the assessment of prognosis. The prognosis for lymphoma from B-lymphocytes of the germinal center of the follicle is more favorable than for lymphoma from activated B-lymphocytes

Loading...Loading...