Lgm symptoms. Lymphogranulomatosis: symptoms. Symptoms due to damage to internal organs

Lymphogranulomatosis (Hodgkin's disease) is pathological process, which is characterized by the formation of a malignant formation of lymphoid tissue. This neoplasm has a specific histological structure. Lymphogranulomatosis is most often found in children and in patients aged 20–50 years.

What influences the formation of the disease?

The exact reasons contributing to the development of the disease are unknown. Although Hodgkin's lymphoma became known to people back in 1832. According to many experts, the causes of the disease may be hidden in the Epstein-Barr virus. The causative agent of the virus is often found in neoplasm cells. Scientists believe that it is these kinds of reasons that contribute to the formation of lymphoid mutations, as a result of which their number rapidly increases. Often the causes of Hodgkin's lymphoma lie in a hereditary factor.

Stages

Hodgkin's disease can have four stages:

1. The first stage (local) – one zone of the lymph nodes is affected.
2. The second stage - various areas of the lymph nodes may also be involved in the process. In addition, the second stage is accompanied by simultaneous damage to one extralymphatic tissue located on the same side of the diaphragm.
3. The third (generalized) stage - Hodgkin's disease affects the lymph node zones in a single or plural on both sides of the diaphragm.
4. Fourth (disseminated) stage - the pathological process affects one or more extralymphatic tissues or organs with simultaneous damage to the lymph nodes.

How to recognize acute and subacute lymphogranulomatosis?

Hodgkin's lymphoma in acute form can be recognized by the following symptoms:

1. A sharp rise in temperature, which is wave-like. It can rise sharply and then fall. This condition is accompanied by drenching cold sweats.
2. Hodgkin's lymphoma in the acute phase causes headaches, pain syndrome in muscles and joints.
3. General weakness poor appetite, rapid weight loss.
4. Hodgkin's lymphoma causes enlargement of one or more groups of lymph nodes. The most affected areas are the cervical and submandibular lymph nodes. They are characterized by elasticity, dense to the touch and do not cause pain at all.
5. Enlarged spleen and liver.

There are cases when the symptoms of lymphogranulomatosis begin to manifest themselves with high fever, general weakness, weight loss, but enlargement of the lymph nodes occurs later. Hodgkin's disease progresses rapidly and becomes diffuse. The result of this process can be death due to various complications.

Complications of Hodgkin's lymphoma in acute form are: pulmonary heart failure, pulmonary edema, renal and liver failure. For this reason, it is important to treat Hodgkin lymphoma promptly.

If there is a acute course, then Hodgkin’s disease manifests itself less acutely and rapidly, so the consequences occur in a reduced amount.

How to recognize chronic diseases?

In chronic Hodgkin's lymphoma, symptoms develop slowly and unnoticed. Hodgkin's disease can be recognized by the following signs:

1. An increase in the size of one lymph node (inguinal, cervical, submandibular). The enlargement is small, the lymph node itself is small, dense and does not cause pain. After some time, it may completely disappear and appear again.
2. Tumor cells of Hodgkin lymphoma are concentrated in nearby lymph nodes. As a result, they also enlarge and resemble sacks of potatoes.
3. Fatigue, bad dream and appetite.
4. Headache.
5. Increase in body temperature.

The chronic course of Hodgkin's lymphoma can occur completely unnoticeably. Wherein general state the patient is not impaired at all, and he can carry out all his usual activities.

Symptoms of lymphogranulomatosis of bones

If Hodgkin's lymphoma affects the lymph nodes, then the bones located next to them also undergo changes. When the vertebrae are affected, compression of the nerve roots is observed. This process is manifested by local or radiating pain. If you press on the affected areas, the pain only intensifies. If the ribs and sternum are involved in the process, then Hodgkin's disease manifests itself in the form of pain and tumor formation in the affected area. The pathological process affecting the pelvic bones is accompanied by the germination of tumors from the pelvic iliac lymph nodes. Rarely, damage to the pelvic bones is caused through the blood.

Manifestations of lymphogranulomatosis of the gastrointestinal tract

If Hodgkin's disease affects the gastrointestinal tract, then the first stage of the disease does not give any manifestations. When certain changes occur in the organs, the patient begins to feel certain symptoms. When Hodgkin's lymphoma forms in the stomach and duodenum, symptoms characteristic of peptic ulcer: flatulence, intestinal colic and diarrhea.

The chronic form of Hodgkin lymphoma can last for a long time, after which its activity subsides. This disease cannot be treated, unlike the acute and subacute types of the course.

Classification of the disease

You can confirm the presence of Hodgkin lymphoma only after the doctor analyzes a tissue sample lymph node when performing a biopsy. Highlight the following types presented pathology:

1. Nodular sclerosis.
2. Mixed cell.
3. Lymphoid depletion.
4. Nodular sclerosis with a pronounced predominance of lymphocytes.

Nodular sclerosis is the most common diagnosis that doctors give to people who suspect lymphogranulomatosis. The disease most often affects women and is accompanied by damage to the lower cervical, supraclavicular and mediastinal lymph nodes. Nodular sclerosis can affect young people and has a good prognosis. However, nodular sclerosis can take chronic form which lasts for many years.

Lymphogranulomatosis venereum

Inguinal lymphogranulomatosis is a chronic infectious disease, which is transmitted through sexual contact, and is accompanied by damage to the soft tissues of the urogenital area and lymph nodes.

The length of the incubation period can vary greatly. Here everything depends on factors such as the cause of the disease, treatment of concomitant diseases, and the number of pathogens that have died.

Often lymphogranulomatosis venereum lasts 3–7 days, but there have been cases when lymphogranulomatosis inguinal is bothersome human body within 12 weeks. When the incubation period is over, the patient diagnosed with lymphogranulomatosis venereum complains of general malaise, weakness, fever and pain in the head.

Inguinal lymphogranulomatosis can occur in three stages:

1. The first stage is when the first symptoms appear on the affected area.
2. The second is that against the background of a general reaction of the body, lymphogranulomatosis venereum affects peripheral lymph nodes.
3. Third, lymphogranulomatosis venereum is accompanied by severe manifestations. In the anal area, on the mucous membrane of the rectum and genital organs, inflammatory, ulcerative and hypertrophic processes have formed.

Children's lymphogranulomatosis

Hodgkin's lymphoma most often affects young patients preschool age. Boys suffer from this disease 2 times more often than girls. Lymphogranulomatosis in children is accompanied by the following manifestations:

• enlarged lymph nodes;
• fever;
• increased sweating at night;
• fast fatiguability;
• weight loss;
• drowsiness;
• poor appetite;
• itching skin.

Diagnostics

To recognize Hodgkin's disease, which contributes to enlarged lymph nodes, it is necessary to involve the following diagnostic methods in the process:

1. Opening chest And abdominal cavity.
2. X-ray studies.
3. Method of scanning retroperitoneal lymph nodes.

To clarify the stage of lymphogranulomatosis - Hodgkin's disease, you need to conduct the following studies:

• medical examination;
• chest x-ray;
• percutaneous bone marrow biopsy;
• liver, spleen and radionuclide scans;
• contrast angiography.

Blood analysis

A blood test is a type of research that allows you to accurately and quickly determine changes in the composition of peripheral blood. Such changes in the blood test confirm the presence of a tumor process and its complications. Analysis in progress cellular composition the patient’s blood, the shape and size of each type of cell is assessed.

Hodgkin's lymphomas do not cause the formation of specific changes in the blood, so that the diagnosis of the disease could be confirmed. For this reason general analysis blood tests are prescribed to accurately determine the condition of various organs and systems of the body.

Blood is drawn in the morning on an empty stomach. Before taking a blood test, you need to refrain from severe physical complications, therapy and drinking alcohol. If possible, then intramuscular injection medications should also be postponed. A general blood test involves donating capillary and venous blood.

Hodgkin's disease can be diagnosed using a biochemical blood test. The presence of any pathological process is accompanied by an increase in protein concentration. Therefore, this analysis makes it possible to determine proteins of the acute phase of inflammation and liver tests.

Therapy

Today, treatment of lymphogranulomatosis is carried out successfully until the patient fully recovers. The determining factors for successful recovery from Hodgkin lymphoma are the stage of the disease and timely seeking medical help.

Treatment of lymphogranulomatosis at the first and second stages gives 100% positive result, however, in the final stages the situation is not so simple. Hodgkin's lymphoma and its manifestations can be eliminated using the following therapeutic measures:

1. Radiation therapy.
2. Chemotherapy. The doctor may prescribe such treatment when Hodgkin lymphoma occurs in the second and third stages.
3. High-dose chemotherapy with autologous bone marrow transplant.

Therapy in children

Lymphogranulomatosis in children is treated in a hospital setting. The number of therapeutic measures is determined by the total clinical picture illness. Today, the maximum effect is achieved when using complex therapy: combined use of radiation treatment and various chemotherapy regimens. Thanks to such manipulations, it is possible to improve long-term results. Side effects must be taken into account when treating Hodgkin's lymphoma in this manner. Radiation therapy can cause skin damage, internal organs.

Forecast

The prognosis of lymphogranulomatosis is favorable if the disease proceeds without relapse. Hodgkin's lymphoma with supradiaphragmatic tumor localization leaves the human body in 90% of cases with complex treatment. With the third stage of Hodgkin's lymphoma, the survival rate is 80%, and with the fourth - 45%.

Preventive actions

Not developed yet effective prevention Hodgkin's disease - lymphogranulomatosis. Usually, Special attention focused on relapses. In this case, compliance with the required regime and rhythm is indicated. Everyday life. The disease reoccurs during insolation and pregnancy. A woman can become pregnant after suffering from an illness 2 years from the moment remission occurs.

Hodgkin's lymphoma is a serious disease, the main manifestation of which is enlarged lymph nodes. This pathology can occur not only in adults, but also in children. Only an integrated approach to treatment will eliminate all its manifestations and return to your previous life.

22.06.2017

Lymphogranulomatosis is a malignant disease in which granulomas, pathological cells from lymphoid tissue, are formed.

The disease begins with damage to some lymph nodes, then the tumor process spreads to other organs (liver, spleen, etc.).

The disease is very rare, accounting for about 1% of the total number of cancer cases. The second name for lymphogranulomatosis is Hodgkin's disease, named after the doctor who first described it. Hodgkin's lymphoma occurs in 2-5 cases per million people per year. The peak incidence occurs in the age groups: 20-30 years and after 50 years. The disease is common in tropical countries. Men get sick more often (1.5-2 times) than women. It ranks second among hemoblastoses in terms of frequency.

Sometimes the disease occurs in an acute form with bright and painful signs, and this course of the disease quickly leads to the death of a person. Most common (90%) chronic course lymphogranulomatosis, with periods of exacerbation and remission, but the disease quickly affects life important organs and has disappointing forecasts.

Causes

Among the causes of the disease today are:

• heredity (genetic predisposition);
• viral origin, autoimmune diseases;
• immune disorders (immunodeficiency).

In T-cell immunodeficiency, the main sign of lymphogranulomatosis is the presence of multinuclear giant cells (Berezovsky-Reed-Sternberg), as well as their prestages - mononuclear cells (Hodgkin) in the lymphatic tissue. Pathological tissues contain eosinophils, plasma cells, and histiocytes. Although the etiology of the disease has not been fully studied by doctors.

Other factors that provoke any oncology are also possible: radiation, work in hazardous enterprises (chemical, woodworking, agricultural), nutrition, bad habits.

Tumor degeneration of lymphocytes is provoked by viruses, most often herpes (Epstein-Barr virus), which affects and leads to rapid division and destruction of B-lymphocytes. In 50% of patients, this virus was found in tumor cells.

Persons with AIDS are predisposed to lymphogranulomatosis. In an immunodeficient state, antitumor protection decreases and the risk of developing lymphogranulomatosis increases.

Doctors observe a genetic predisposition to this disease, for example, the pathology is often found in twins and family members. However, the specific gene that increases the risk of lymphoma has not been identified.

People who take medications after an organ transplant or when treating other tumors are more at risk of developing Hodgkin's lymphoma, since these drugs lower the immune system.

90% of the population has Epstein-Barr virus, but only 0.1% of people develop the disease.

Symptoms of lymphogranulomatosis

Lymphogranulomatosis is accompanied by the following symptoms:

• enlarged lymph nodes;
• symptoms of organs affected by the disease;
• systemic manifestations of the disease.

The first signs of lymphogranulomatosis are enlarged lymph nodes (one or more). Initially, cervical and submandibular or axillary and inguinal. The lymph nodes are still painless, but greatly enlarged (sometimes to enormous sizes), densely elastic, not fused to the tissues, and mobile (move under the skin). Dense scar cords grow in the lymph nodes and a granuloma forms.

In 60-80% of cases, the cervical (sometimes subclavian) lymph nodes are the first to be affected, in 50% of cases – the mediastinal lymph nodes. From the primary site, the tumor metastasizes to important organs (lungs, liver, kidneys, gastrointestinal tract, bone marrow).

General symptoms that concern the patient: excessive sweating at night, periodic increases in temperature (up to 30 ° C) and fever, noticeable weight loss, weakness, itching.

Explicit and more tangible symptoms “B”, characteristic of a progressive form of lymphogranulomatosis, require treatment:

• general loss of strength, loss of strength;
• chest pain, cough;
• disruption of the gastrointestinal tract, indigestion;
• stomach ache;
• ascites (accumulation of fluid in the abdomen);
• joint pain.

Difficulty breathing and cough, which gets worse when lying down, are caused by compression of the trachea by enlarging lymph nodes. A constant feeling of exhaustion, loss of appetite and other symptoms listed above should force a person to consult a doctor without delay.

Diagnosis of lymphogranulomatosis

To determine lymphogranulomatosis, diagnostics will be carried out using general clinical tests, instrumental and biochemical methods. The examination results help to assess the condition of the liver and spleen and determine the degree of development of the disease. To make a diagnosis, chest X-rays, CT scans, and MRIs are performed to assess the extent of damage to other important organs.

A blood test may show anemia, thrombocytopenia, increased ESR, sometimes Berezovsky-Sternberg cells and pancytopenia (a decrease below normal levels of all blood elements) are detected, which indicates advanced disease.

A biochemical blood test may show excess bilirubin and enzymes, which indicates damage to the liver, globulins, and seromucoids.

X-ray in early stage The disease will show the degree of enlargement of the lymph nodes. CT scans showing the growth of lymph nodes in all human organs and makes it possible to clarify the stage of the process. MRI gives similar results and makes it possible to determine the location of the tumor and its size.

When a diagnosis is made, adequate treatment is prescribed, necessary for a particular patient, depending on the stage of the disease.

Treatment of lymphogranulomatosis

At stages 1 and 2, radiation therapy is indicated. It can give stable remission, but is associated with serious complications. To avoid negative effects on healthy tissue, leading experts recommend against extensive radiation therapy. And carry out targeted radiation on the tumor after a course of chemotherapy.

Chemotherapy involves taking medicines to destroy cancer cells. These cytotoxic drugs affect both the tumor and the entire body. Patients who have undergone chemotherapy require long-term medical supervision to monitor its long-term consequences.

2 standard chemotherapy regimens are used:

1. ABVD (abbreviation of 4 drugs included in it) - adriamycin, bleomycin, inblastine, dacarbazine.
2. Stanford V (7 drugs) - adriamycin, bleomycin, vinblastine, vincristine, mechlorethamine, prednisolone, etoposide.

Patients undergo 4-8 courses depending on the stage of the disease. The second scheme is used when there is a high risk of malignant development of the process. Maximally effective, positive results are confirmed in 95% of patients. This method is dangerous due to the risk of secondary cancers, which may be a consequence of chemotherapy. In case of relapse, a second course of chemotherapy and a bone marrow transplant are prescribed.

Lymphogranulomatosis responds well to treatment, especially in the first stages of the disease. And in later stages, the survival rate is higher than with other types of oncology.

Lymphogranuloma venereum

A type of disease - lymphogranulomatosis venereum (lymphogranuloma venereum) is transmitted sexually and very rarely through household contact. This type of lymphogranuloma is common in Africa, South America, Africa and port cities and is extremely rare in Russia.

5-21 days (incubation period) after infection, a seal (tubercle or vesicle) appears on the genitals and disappears, often going unnoticed.

Then the lymph nodes thicken and enlarge: inguinal nodes in men, in the pelvic area in women. The lymph nodes soon become welded together, become painful and greatly increase in size, and spontaneously break through the skin. Pus is released from the wounds. To make a diagnosis of lymphogranuloma venereum, a study is carried out: culture, ELISA, RIF.

Treatment

How to treat lymphogranulomatosis venereum? Both sexual partners should be treated, even if one is sick, to prevent re-infection.

If there is a risk of infection for prevention, you can undergo treatment during the incubation period to prevent a disease that threatens serious complications - the occurrence of fistulas (in anus, vagina, scrotum), later narrowing of the rectum or urethra and elephantiasis (enlargement, swelling) of the genitals.

Inguinal lymphogranulomatosis

The “fourth” venereal disease is called inguinal lymphogranuloma or inguinal lymphogranulomatosis. It is common in the tropics, and mainly tourists visiting exotic Asian, South American and African countries are at risk of infection. This disease is therefore also called “tropical bubo”. It was first described by French doctors in the 19th century.

The causative agent is a serotype of chlamydia trachomatis, an STI (sexually transmitted infection), the same bacterium that causes chlamydia. Chlamydial lymphogranuloma can be infected through sexual contact, as well as through the use of household and intimate objects of a virus carrier. Carriers of the virus with an asymptomatic course of the disease are dangerous in terms of infection. The disease can occur in acute and chronic form.

Bacteria, getting on the mucous membrane of the intimate organs or mouth, on the fingers, can easily spread to other organs. The risk group is young people (20-40 years old), symptoms appear more quickly in men.

The incubation period ranges from several days to 4 months. At this moment, the disease does not manifest itself in any way. Inguinal lymphogranulomatosis occurs in 3 stages.

During the first stage, a blister appears at the site of infection, which eventually matures into an ulcer. The outlines of a purulent ulcer are round, measuring -1-3 cm. Unlike syphilis, the ulcer is without compaction at the base, with an inflamed rim. After a week, the pelvic lymph nodes enlarge in women, and the inguinal lymph nodes in men.

The second stage (after 2 months) is characterized by increased inflammation, large enlargement of lymph nodes with spread inflammatory process to nearby tissues. Fistulas with purulent discharge appear in the tumor. The adhesions that appear disrupt the lymph flow, which is accompanied by swelling of the genital organs.

At this stage, the deep lymph nodes are also affected, the infection penetrates into the internal organs, joints, and limbs. Skin manifestations (urticaria, eczema) may appear. Necrotic changes in the lymph nodes may occur, which will be clearly reflected in the blood test results.

Symptoms of damage to the liver, spleen, and eye diseases are accompanied by high temperature, fever, and joint pain.

Inguinal lymphogranulomatosis in the third stage, if treatment is not carried out, severe consequences of the disease are observed: the perineum, rectum, and surrounding tissues become inflamed. Abscesses, ulcers, fistulas, and erosions are localized in them, from which blood and pus are released. The patient loses a lot of weight, suffers from headaches and polyarthritis. He has no appetite, general weakness and anemia.

Diagnostics

Using laparotomy, the presence of pathological cells in the lymph nodes is first examined. If the stage of the disease is complex and other organs may be affected, additional examination is performed. Carry out:

• examination of the liver, spleen;
• chest x-ray;
• A bone marrow biopsy is taken.

Usually stages 1 and 2 are accompanied by such symptoms that a person is forced to consult a doctor. The disease cannot be brought to the third (severe) stage, when some consequences and complications are already irreversible.

Treatment of inguinal lymphogranulomatosis

During treatment, antibiotics are used to destroy the causative agent of the disease, and measures are taken to avoid cicatricial deformities. Immunomodulators are prescribed to strengthen the body's defenses.

No self-medication or folk remedy will not help. The infection destroys health by penetrating important body systems. Treatment should be prescribed only by a doctor, taking into account the condition of the individual patient and the stage of development of the disease. The course of treatment is about 3 weeks. The patient should be under the supervision of the attending physician for some time to finally make sure that the disease has subsided.

In severe and advanced cases, it is sometimes necessary to resort to the help of surgeons.

Modern medicine has enough effective methods and medications to successfully defeat the disease. Lymphogranuloma venereum is easily cured effective techniques treatment For successful treatment and full recovery, be attentive to your health and seek help from doctors in a timely manner.

Prevention

To avoid the spread of STDs, doctors not only treat the sick person, but try to establish the circle of his sexual partners in order to prevent the disease or provide timely treatment.

It is very important to visit a venereologist and get tested if you are planning to have a child, have returned from a trip to tropical countries, or if you are not sure about casual sexual contact.

Avoid promiscuity, use condoms during sex, and do not share other people's hygiene products to avoid STIs.

Symptoms of lymphogranulomatosis in blood tests are one of the simplest and most reliable methods for preliminary diagnosis of Hodgkin lymphoma.

The main soldiers of human immunity are lymphocytes. This is one of the types of leukocytes, thanks to which people either do not get sick or get better.

IN leukocyte formula In adults, the number of lymphocytes is at least a quarter; in children, this figure reaches 50%.

Lymphocytes produce antibodies to various pathogens, and also take part in cellular immunity, destroying cells that do not meet the standard, for example, malignant ones.

The lymphatic system consists of nodes united into a common network of vessels. The neoplastic (malignant) process in this system is called lymphoma.

Lymphocytes degenerate and divide uncontrollably, the process gradually spreads throughout the body, affecting various organs.

Lymphoma is not one disease, but a whole group of about thirty varieties. Each lymphoma has proper name, since it differs significantly in course, prognosis and treatment.

The most general classification allows us to divide all lymphomas into Hodgkin lymphoma (lymphogranulomatosis) and non-Hodgkin lymphomas.

Specific symptoms of lymphogranulomatosis and their difference from non-Hodgkin lymphomas are Reed-Berezovsky-Sternberg cells. These giant cells can be seen in microscopic examination of a biopsy specimen.

Hodgkin's lymphoma - a name introduced in 2001 World Organization health (WHO). Other names: Hodgkin's disease, lymphogranulomatosis, malignant granuloma.

Back in the first half of the 19th century, the disease was studied by the British physician Thomas Hodgkin. He observed seven patients with enlarged lymph nodes, liver and spleen, bringing this disease to the attention of the scientific community for the first time.

Hodgkin's patients died because the disease was incurable at the time, but modern medicine makes great strides in overcoming the disease.

The disease occurs in both children and adults; the vast majority become ill between the ages of 15 and 40 years.

According to statistics, the disease affects 2.3 people per 100 thousand population. A slight predominance of cases is among men (adults and children).

The causes of the disease are unknown, but scientists are inclined to exclude a hereditary factor, since cases of lymphogranulomatosis in one family are rare.

However, some types of lymphoma occur with surprising frequency in people, including children with the most common virus, Epstein-Barr virus.

Types of Hodgkin's lymphoma and diagnosis of the disease

Studying a biopsy of the affected tissue for the presence of Reed-Berezovsky-Sternberg cells is a mandatory point in the diagnosis of lymphogranulomatosis.

Many scientists believe that it is these giant ones that are tumorous, and that other cellular changes and fibrosis arise as a response immune system to a malignant process.

Depending on the biopsy picture, four types of lymphogranulomatosis are distinguished.

Lymphohistiocytic lymphogranulomatosis accounts for about 15% of all cases of the disease. It usually affects men under 35 years of age.

The variety is characterized by a large number of mature lymphocytes with a low prevalence of Reed-Berezovsky-Sternberg cells.

This variant of the disease is considered low-grade and if detected early, treatment has a good prognosis.

The type with nodular sclerosis is more widespread than others, accounting for 40–50% of patients, mostly young women.

The disease is usually localized in the mediastinal lymph nodes and has a good prognosis. The main characteristic is the combination of Reed-Berezovsky-Sternberg cells with lacunar cells.

The mixed cell variety accounts for about 30% of cases of lymphogranulomatosis.

As a rule, the disease is typical for residents of developing countries, and mainly occurs in children or older people (boys and men).

The cells are characterized by polymorphism (diversity), among them there are many Reed-Berezovsky-Sternberg cells.

Lymphogranulomatosis, which suppresses lymphoid tissue, is a rare type, occurring in no more than 5% of cases. Most of the patients are elderly; the disease does not occur in children.

The biopsy sample contains mainly Reed-Berezovsky-Sternberg cells, and no lymphocytes at all.

Diagnosis of lymphogranulomatosis in children and adults includes a number of mandatory methods.

These include a physical examination for enlarged lymph nodes, taking a detailed medical history, especially to identify characteristic symptoms(over the last six months):

• unreasonable weight loss of more than 10%;
• feverish conditions at body temperature up to 38 °C;
• profuse sweating.

Diagnosis also includes surgical biopsy and bone marrow biopsy, laboratory research blood (general and biochemical analysis), myelogram, chest x-ray.

Hodgkin's lymphoma in blood tests

Changes in blood tests with lymphogranulomatosis in children and adults are nonspecific, that is, similar symptoms are characteristic of a number of other diseases.

However, various symptoms taken together (blood counts, medical history, physical examination findings) suggest lymphogranulomatosis, which can be definitively confirmed or refuted by further diagnostics aimed at specific symptoms.

However, due to its simplicity, accessibility and high information content, clinical and biochemical blood tests are a mandatory stage of preliminary diagnosis.

CBC and blood biochemistry studies definitely do not provide information about the type and variety of lymphoma and, accordingly, they cannot be used to determine a treatment protocol, although preliminary ideas about the prognosis can be obtained.

Symptoms in a blood test for lymphogranulomatosis:

In a biochemical blood test for the diagnosis of lymphogranulomatosis in children and adults, the following are most indicative:

1. proteins characteristic of acute inflammatory processes;
2. liver tests.

An increase in the amount of proteins in the blood is the body’s response to a pathogenic process.

With lymphogranulomatosis, proteins increase tens and sometimes hundreds of times, they neutralize toxic effect tumor cells, and their number indicates the degree of the inflammatory process.

Liver tests allow you to determine the degree of liver damage, which has a greater load in case of lymphogranulomatosis. The closer the indicators are to normal, the better the treatment prognosis.

Doctor's opinion

TomoClinic Clinical Oncologist Fadel Olaki says:

“Hodgkin's lymphoma is one of the first cancers to be successfully treated for a large group of patients around the world. Thanks to the modern TomoTherapy® system installed in our clinic, we treat even the most severe forms of Hodgkin lymphoma with complex localizations. Irradiation on our device is carried out simultaneously for several zones and we manage to maintain an individual dose for each of them.”

What is lymphogranulomatosis?

Hodgkin's lymphoma(synonyms: lymphogranulomatosis, Hodgkin's disease) is a tumor disease of the lymphatic system. The pathology accounts for approximately 1% of all cancers registered annually in the world. Lymphogranulomatosis can manifest itself at any age, but there are two peaks of the disease: the first occurs at the age of 20-29 years, the second at 60 years and older.

Lymphogranulomatosis is a disease that, as already mentioned, affects the human lymphatic system. The oncologist can fully confirm the diagnosis if the neoplasms contain so-called Berezovsky-Sternberg-Reed giant cells, which “inform” the doctor about an anomaly in the normal functioning of the tissues.

Hodgkin's disease is a malignant tumor, but is it cancer or not? From a medical point of view, this neoplasm is not included in the group of cancer pathologies, since the systemic nature of the lesion is not consistent and the treatment of the disease is quite simple. Men are more susceptible to developing pathology than women. Berezovsky-Sternberg-Reed tumor cells can only be detected by specialists as a result of examining the patient after taking a biochemical blood test. This is why it is so important to contact a good medical Center.

Lymphogranulomatosis - causes

The reasons for the development of lymphoma have not yet been fully identified. It has been established that patients with immunodeficiency conditions are much more likely to develop the disease. Epstein-Barr virus is found in 40-60% of human cases of Hodgkin's disease. Based on this fact, a certain role of the virus in the development of lymphoma is considered.

Lymphomas are not a hereditary disease; transmission from person to person is not possible. Lymphoma can also rarely be caused by congenital genetic defects. The etiology is not reliably understood, but in medicine there is evidence of increased incidence among residents of certain regions, which makes it possible to draw conclusions about the influence of viruses and external factors on the incidence rate among the population.

Among the predisposing factors, doctors identified the following:

1. Heredity (if one of your relatives has been diagnosed with pathologies of lymphoid tissue, then there is a high probability of developing this pathology).
2. Immunodeficiency from birth or acquired during life.
3. Autoimmune pathologies, for example, lupus erythematosus.

There is a close connection between infectious mononucleosis and the development of Hodgkin's disease. However, this pathology does not develop in all people who have the virus in their blood. According to ongoing research, the isolated impact of this factor determines the probability of developing pathology at only 0.1%. At the same time, the Epstein-Barr virus is statistically detected in 90 percent of the world's population. The risk group also includes people with various types of immunodeficiency conditions, for example those who have been diagnosed with HIV infection.

Symptoms of lymphogranulomatosis

Symptoms of the pathology manifest themselves in different ways: the disease begins its development in the lymph nodes, and the further painful process can spread to almost all organs, often accompanied by variously expressed intoxication manifestations. The picture of lymphogranulomatosis is determined by the location of the tumor and the damage to one or another organ.

The first sign of pathology is enlarged lymph nodes. In 60-75% of cases, the oncological process begins in the cervical-supraclavicular lymph nodes, and is most often affected Right side. Usually, enlarged lymph nodes do not lead to deterioration in health and the person does not notice any changes. In 15-20% of cases, the mediastinal lymph nodes are initially affected. It may be discovered incidentally during an X-ray examination or when lymph node enlargement becomes significant. In the second case, patients turn to medical institution with complaints of cough, shortness of breath, swelling and cyanosis of the face, and sometimes chest pain.

In isolated cases, lymphogranulomatosis begins with damage to the para-aortic lymph nodes; the patient may experience back pain that worsens at night. In 5-10% of patients, lymphogranulomatosis begins with fever, weight loss, night sweats, and itching occurs in 20-25% of patients. With further development of lymphogranulomatosis, damage to all lymphoid and other systems and organs of the body is possible. In addition to the lymph nodes, Hodgkin lymphoma most often affects bones and lung tissue. Involvement of the lungs in the process may not manifest itself clinically, but pain appears when bones are damaged. Along with local symptoms, the patient is seriously tormented general manifestations(group B symptoms):

1. Feverish state ( heat body), which does not go away for more than 7 days.
2. Violent sweating during night sleep.
3. Uncontrolled weight loss (more than 10 percent weight loss within six months).

Symptoms “B” characterize the most serious course of the pathology and make it possible to determine the need for intensive treatment. Among other symptoms characteristic of this disease are:

1. A state of weakness, no desire to take any food, complete loss of strength.
2. The skin is very itchy.
3. Cough, chest pain, difficulty breathing.
4. Pain syndrome in the peritoneum, digestive system disorder.
5. Ascites.
6. Pain syndrome in bone tissue.
7. Enlarged lymph nodes.

In some cases, the first symptoms dangerous pathology during long term manifested by a constant feeling of lethargy and rapid fatigue. Breathing problems develop when the lymph nodes inside the sternum become enlarged. As the nodes grow and damage to the body continues, they begin to put pressure on the trachea, provoke the development of cough and other problems with respiratory system. These symptoms become more pronounced when the person is lying down. Sometimes a person may feel pain behind the chest.

It is important to understand: as soon as the first symptoms of Hodgkin lymphoma have developed (preferably before the lymph nodes have enlarged and damage to internal organs has begun), you need to visit a good medical center, where the doctor will conduct comprehensive diagnostics and pick up effective method treatment.

Classification of Hodgkin's lymphoma

In hematology, a local form of lymphogranulomatosis is distinguished. During an isolated pathological process, one group of lymph nodes is affected. A generalized form with low-quality proliferation in the following internal organs and systems is also distinguished:

1. Lungs.
2. Stomach.
3. Liver.
4. Spleen.
5. Skin.

Based on location, the following types of pathology are distinguished:

1. Pulmonary.
2. Bone.
3. Skin.
4. Mediastinal
5. Abdominal.
6. Nervous.
7. Gastrointestinal Hodgkin's disease.

Depending on the speed of development of the disease, lymphogranulomatosis can occur acutely (several months from the onset of the pathological process to the terminal stage). A chronic course of the disease can also be observed (protracted, long-term with periodic cycles of exacerbations and subsidence of the pathology). Based on the morphological method of studying the formation and quantitative relationships of various types cellular elements In medicine, there are 4 histological forms of Hodgkin's disease:

1. Lymphoid or lymphohistiocytic.
2. Nodular sclerotic or nodular sclerosis.
3. Mixed cell.
4. Lymphoid depletion.

Only qualified specialists can determine the presence of a serious disease and classify the pathology.

Lymphogranulomatosis: stages

The basis clinical classification Hodgkin's disease is a criterion for the prevalence of a malignant process. In accordance with it, the development of lymphogranulomatosis goes through four stages:

1. Local stage (first): one group of lymph nodes (I) or one extralymphatic organ (IE) is affected.
2. Regional stage (second): 2 or more groups of lymph nodes are affected, which are located on one side of the diaphragm (II) or one extralymphatic organ and its regional lymph nodes (IIE).
3. Generalized stage (third): affected lymph nodes, which are located on both sides of the diaphragm (III). Additionally, one extralymphatic organ (IIIE), the spleen (IIIS), or both (IIIE + IIIS) may be affected.
4. Disseminated stage (fourth): one or more extralymphatic organs are affected (lungs, pleura, bone marrow, liver, kidneys, gastrointestinal tract and others) with or without simultaneous damage to the lymph nodes.

To indicate the presence or absence of general symptoms of Hodgkin's disease for six months (fever, night sweats, weight loss), the letters A or B are added to the number that indicates the stage of the disease, respectively.

Diagnosis of lymphogranulomatosis

Most often, lymphomas are discovered incidentally. To make a diagnosis of lymphogranulomatosis, it is important to carefully study the history of the development of the disease, symptoms of the disease with an assessment of the condition of all lymph nodes accessible to palpation. Biopsy of the affected lymph node followed by histological examination- diagnostic standard.

The diagnosis of Hodgkin's lymphoma is established only when specific multinucleated Berezovsky-Sternberg cells are detected in the affected lymph node. In doubtful cases, immunophenotyping is necessary. If lymphogranulomatosis is suspected, in addition to a lymph node biopsy, blood parameters are assessed (biochemical blood test, general blood test), radiation diagnostics, and, if necessary, bone marrow puncture.

Modern medical equipment (CT, MRI and others) makes it possible to detect lymphogranulomatosis tumors in the early stages of development. Also an important factor is the competence of the specialist who will conduct the examination.

Treatment of lymphogranulomatosis

Treatment of lymphogranulomatosis is prescribed after obtaining the necessary results of laboratory and clinical studies, implying the use of modern highly effective techniques. Hodgkin's lymphoma was one of the first oncological diseases in which the possibility of curing a large group of patients was shown. When treating lymphogranulomatosis, chemotherapy and radiation therapy are combined.

Innovative solutions at TomoClinic

High-quality diagnostics, staging and treatment of this type of oncology are carried out at TomoClinic. During the treatment process, the patient remains in the medical center under the constant supervision of specialists. TomoClinic is the first and only medical center in Ukraine that uses the latest effective type radiation therapy(tomotherapy). The TomoTherapy® system allows you to successfully treat even the most severe forms and localizations. Irradiation can be performed simultaneously in several zones, maintaining an individual dose for each of them.

Radiation therapy treatment

During a course of radiation therapy, the radiation dose is delivered only to the pathological formation. All adjacent tissues and nearby organs remain safe during treatment. The procedure does not cause pain or psychological discomfort. Our clinic is equipped modern equipment For complex treatment almost all types malignant tumors. We employ the best specialists in Ukraine, for whom not only the positive result of treatment for lymphogranulomatosis is important, but also the patient’s quality of life after therapy. TomoClinic does not accept compromises in the fight against cancer, and the Tomo HD device is rightfully considered revolutionary in the world of radiation therapy. Best clinics all over the world are equipped with this system, and now it is also in Ukraine.

To verify the diagnosis, a lymph node biopsy is performed, diagnostic operations(thoracoscopy, laparoscopy), chest radiography, ultrasound, CT, bone marrow biopsy. For therapeutic purposes, lymphogranulomatosis is treated with polychemotherapy, irradiation of the affected lymph nodes, and splenectomy.

Lymphogranulomatosis

Lymphogranulomatosis (LGM) is a lymphoproliferative disease that occurs with the formation of specific polymorphic cell granulomas in the affected organs (lymph nodes, spleen, etc.). After the name of the author who first described the signs of the disease and proposed to isolate it into an independent form, lymphogranulomatosis is also called Hodgkin's disease, or Hodgkin's lymphoma. The average incidence of lymphogranulomatosis is 2.2 cases per 100 thousand population. Among the sick people, young people predominate; the second peak incidence occurs at ages over 60 years. In men, Hodgkin's disease develops 1.5-2 times more often than in women. In the structure of hemoblastoses, lymphogranulomatosis is given three times the place in frequency of occurrence after leukemia.

Causes of lymphogranulomatosis

The etiology of lymphogranulomatosis has not yet been clarified. Today, viral, hereditary and immune theories of the genesis of Hodgkin's disease are considered among the main ones, but none of them can be considered exhaustive and generally accepted. The possible viral origin of lymphogranulomatosis is supported by its frequent correlation with previous infectious mononucleosis and the presence of antibodies to the Epstein-Barr virus. At least 20% of the Berezovsky-Sternberg cells tested contain genetic material from the Epstein-Barr virus, which has immunosuppressive properties. The etiological influence of retroviruses, including HIV, cannot be ruled out.

The role of hereditary factors is indicated by the occurrence of the familial form of lymphogranulomatosis and the identification of certain genetic markers of this pathology. According to the immunological theory, there is a possibility of transplacental transfer of maternal lymphocytes into the fetus with the subsequent development of an immunopathological reaction. The etiological significance of mutagenic factors - toxic substances, ionizing radiation, medications and others in provoking lymphogranulomatosis.

It is assumed that the development of lymphogranulomatosis becomes possible in conditions of T-cell immunodeficiency, as evidenced by a decrease in all parts of cellular immunity and a violation of the ratio of T-helpers and T-suppressors. The main morphological sign of malignant proliferation in lymphogranulomatosis (in contrast to non-Hodgkin lymphomas and lymphocytic leukemia) is the presence in the lymphatic tissue of giant multinucleated cells, called Berezovsky-Reed-Sternberg cells and their prestages - mononuclear Hodgkin cells. In addition to them, the tumor substrate contains polyclonal T-lymphocytes, tissue histiocytes, plasma cells and eosinophils. With lymphogranulomatosis, the tumor develops unicentrically - from one focus, often in the cervical, supraclavicular, mediastinal lymph nodes. However, the possibility of subsequent metastasis causes the occurrence of characteristic changes in the lungs, gastrointestinal tract, kidneys, and bone marrow.

Classification of lymphogranulomatosis

In hematology, there is an isolated (local) form of lymphogranulomatosis, in which one group of lymph nodes is affected, and a generalized form - with malignant proliferation in the spleen, liver, stomach, lungs, and skin. Based on localization, there are peripheral, mediastinal, pulmonary, abdominal, gastrointestinal, skin, bone, and nervous forms of Hodgkin's disease.

Depending on the speed of development of the pathological process, lymphogranulomatosis can have an acute course (several months from the initial to the terminal stage) and a chronic course (protracted, long-term with alternating cycles of exacerbations and remissions).

Based on a morphological study of the tumor and the quantitative ratio of various cellular elements, 4 histological forms of lymphogranulomatosis are distinguished:

• lymphohistiocytic, or lymphoid predominance
• nodular sclerotic, or nodular sclerosis
• mixed-cellular
• lymphoid depletion

The clinical classification of lymphogranulomatosis is based on the criterion of the prevalence of the tumor process; According to it, the development of Hodgkin's disease goes through 4 stages:

Stage I (local) – one group of lymph nodes (I) or one extralymphatic organ (IE) is affected.

Stage II (regional) - two or more groups of lymph nodes located on one side of the diaphragm (II) or one extralymphatic organ and its regional lymph nodes (IIE) are affected.

Stage III (generalized) - affected lymph nodes are located on both sides of the diaphragm (III). Additionally, one extralymphatic organ (IIIE), the spleen (IIIS), or both (IIIE + IIIS) may be affected.

Stage IV (disseminated) - the lesion affects one or more extralymphatic organs (lungs, pleura, bone marrow, liver, kidneys, gastrointestinal tract, etc.) with or without simultaneous damage to the lymph nodes.

To indicate the presence or absence of general symptoms of lymphogranulomatosis over the past 6 months (fever, night sweats, weight loss), the letters A or B are added to the number indicating the stage of the disease, respectively.

Symptoms of lymphogranulomatosis

Symptom complexes characteristic of lymphogranulomatosis include intoxication, enlarged lymph nodes and the appearance of extranodal foci. Often the disease begins with nonspecific symptoms - periodic fever with temperature peaks up to 39°C, night sweats, weakness, weight loss, itching.

Often the first “harbinger” of lymphogranulomatosis is an increase in lymph nodes accessible to palpation, which patients discover on their own. Most often these are cervical, supraclavicular lymph nodes; less often - axillary, femoral, inguinal. Peripheral lymph nodes are dense, painless, mobile, not fused to each other, to the skin and surrounding tissues; usually stretch in the form of a chain.

In 15-20% of patients, lymphogranulomatosis debuts with enlargement of the mediastinal lymph nodes. When mediastinal lymph nodes are affected first clinical signs Hodgkin's disease may include dysphagia, dry cough, shortness of breath, and SVC syndrome. If the tumor process affects the retroperitoneal and mesenteric lymph nodes, abdominal pain and swelling of the lower extremities occur.

Among extranodal localizations in lymphogranulomatosis, the most common (in 25% of cases) is lung damage. Pulmonary lymphogranulomatosis occurs as pneumonia (sometimes with the formation of cavities in the lung tissue), and when the pleura is involved, it is accompanied by the development of exudative pleurisy.

In the bone form of lymphogranulomatosis, the spine, ribs, sternum, and pelvic bones are most often affected; much less often - skull bones and tubular bones. In these cases, vertebralgia and ossalgia are noted, and destruction of the vertebral bodies may occur; Radiological changes usually develop after several months. Tumor infiltration of the bone marrow leads to the development of anemia, leukemia and thrombocytopenia.

Lymphogranulomatosis of the gastrointestinal tract occurs with invasion of the muscular layer of the intestine, ulcerations of the mucous membrane, intestinal bleeding. Complications such as perforation of the intestinal wall and peritonitis are possible. Signs of liver damage in Hodgkin's disease include hepatomegaly and increased alkaline phosphatase activity. In case of defeat spinal cord Transverse paralysis may develop within days or weeks. In the terminal stage of lymphogranulomatosis, generalized damage can affect the skin, eyes, tonsils, thyroid gland, mammary glands, heart, testicles, ovaries, uterus and other organs.

Diagnosis of lymphogranulomatosis

Enlargement of peripheral lymph nodes, liver and spleen, along with clinical symptoms (febrile fever, sweating, weight loss) always raises concerns about cancer. In the case of Hodgkin's disease, instrumental imaging methods play a supporting role.

Reliable verification, correct staging and adequate choice of treatment method for lymphogranulomatosis are possible only after morphological diagnosis. In order to collect diagnostic material, a biopsy of peripheral lymph nodes, diagnostic thoracoscopy, laparoscopy, and laparotomy with splenectomy are indicated. The criterion for confirming lymphogranulomatosis is the detection of Berezovsky-Sternberg giant cells in the biopsy specimen. Detection of Hodgkin cells can only suggest an appropriate diagnosis, but cannot serve as a basis for prescribing special treatment.

In system laboratory diagnostics Lymphogranulomatosis requires a complete blood count and biochemical blood parameters to assess liver function (alkaline phosphate, transaminases). If bone marrow involvement is suspected, a sternal puncture or trephine biopsy is performed. At different clinical forms, and also to determine the stage of lymphogranulomatosis, X-rays of the chest and abdominal cavity, CT, ultrasound of the abdominal cavity and retroperitoneal tissue, CT of the mediastinum, lymphoscintigraphy, skeletal scintigraphy, etc. are required.

In the differential diagnostic plan, it is necessary to distinguish between lymphogranulomatosis and lymphadenitis of various etiologies (for tuberculosis, toxoplasmosis, actinomycosis, brucellosis, infectious mononucleosis, tonsillitis, influenza, rubella, sepsis, AIDS). In addition, sarcoidosis, non-Hodgkin's lymphoma, and cancer metastases are excluded.

Treatment of lymphogranulomatosis

Modern approaches to the treatment of lymphogranulomatosis are based on the possibility of complete cure of this disease. In this case, treatment should be step-by-step, comprehensive and taking into account the stage of the disease. For Hodgkin's disease, radiation therapy, cyclic chemotherapy, and a combination of radiation therapy and chemotherapy are used.

As an independent method, radiation therapy is used for stages I-IIA (lesions of single lymph nodes or one organ). In these cases, irradiation may be preceded by lymph node removal and splenectomy. In case of lymphogranulomatosis, subtotal or total irradiation of lymph nodes (cervical, axillary, supra- and subclavian, intrathoracic, mesenteric, retroperitoneal, inguinal) is performed, covering both groups of affected and unchanged lymph nodes (the latter for preventive purposes).

Patients with stages IIB and IIIA are prescribed combined chemoradiotherapy: first, introductory polychemotherapy with irradiation of only enlarged lymph nodes (according to the minimum program), then irradiation of all other lymph nodes (according to the maximum program) and maintenance polychemotherapy over the next 2-3 years.

In disseminated stages IIIB and IV of lymphogranulomatosis, cyclic polychemotherapy is used to induce remission, and at the stage of maintaining remission - cycles drug therapy or radical irradiation. Polychemotherapy for lymphogranulomatosis is performed according to regimens specially developed in oncology (MORR, SORR, SURR, CVPP, DORR, etc.).

The results of the therapy may include:

• complete remission (disappearance and absence of subjective and objective signs of lymphogranulomatosis within 1 month)
• partial remission (relief of subjective symptoms and reduction in the size of lymph nodes or extranodal lesions by more than 50% within 1 month)
• clinical improvement (relief of subjective symptoms and reduction in the size of lymph nodes or extranodal lesions by less than 50% within 1 month)
• lack of dynamics (preservation or progression of signs of lymphogranulomatosis).

Prognosis for lymphogranulomatosis

For stages I and II of lymphogranulomatosis, the relapse-free 5-year survival rate after treatment is 90%; at stage IIIA - 80%, at stage IIIB - 60%, and at stage IV - less than 45%. Unfavorable prognostic signs are the acute development of lymphogranulomatosis; massive conglomerates of lymph nodes more than 5 cm in diameter; expansion of the mediastinal shadow by more than 30% of the chest volume; simultaneous damage to 3 or more groups of lymph nodes, spleen; histological variant lymphoid depletion, etc.

Relapses of lymphogranulomatosis can occur when the maintenance therapy regimen is violated, provoked physical activity, pregnancy. Patients with Hodgkin's disease should be seen by a hematologist or oncologist. Preclinical stages of lymphogranulomatosis in some cases can be detected during preventive fluorography.

Lymphogranulomatosis - treatment in Moscow

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Lymphogranulomatosis, symptoms, treatment, causes, signs

Men get sick somewhat more often than women.

The diagnosis of each variant is established only on the basis of the detection of Sternberg cells. The affected nodes are increased in size, their consistency becomes dense over time.

Causes of lymphogranulomatosis

Depending on the cellular composition of the granuloma, there are 4 histological variants of lymphogranulomatosis:

1. lymphohistiocytic;
2. nodular sclerosis;
3. mixed-cellular
4. lymphoid depletion.

During the course of the disease, transformations of option 1 through the intermediate option 3 to option 4 are possible. Establishing the etiology of lymphogranulomatosis lies in the plane of solving general issues of the etiology of blastoma processes of the hematopoietic system.

Lymphogranulomatosis - rare cancer, the incidence of lymphogranulomatosis in the UK is 3 cases per population. In Western countries, two peaks are noted during the course of the disease, the larger peak occurs in older people, and the smaller peak occurs in old age. In developing countries, cases of the disease are more often reported in children. Representatives of wealthy segments of the population, Caucasians, and those who have had infectious mononucleosis are more predisposed to lymphogranulomatosis. The cause of the disease has not been established and may be different for different histological variants. It has been proven that there is an association between lymphogranulomatosis and carriage of the Epstein-Barr virus, especially in the mixed-cellular variant of the disease and lymphoid depletion in older patients, detected in 30% of cases.

A characteristic diagnostic feature is Reed-Sternberg cells, containing two or more nuclei and surrounded by a cell population, including small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells against a background of fibrotic changes. Infiltrating lymphocytes belong to T lymphocytes, they determine the energetic immune response of the lymph node and possibly promote the survival of tumor cells. Reed-Sternberg cells are malignant cells characteristic of lymphogranulomatosis; according to recent studies, in 97% of cases they belong to B lymphocytes.

The nodular variant of lymphoid predominance is an independent disease, which is a B-cell lymphoma. The latter is characterized by the so-called L&H Hodgkin cells (lymphocytes and histiocytes), according to appearance resembling popcorn (popcorn cells) and expressing the CD20 antigen. In approximately 10% of cases, the nodular variant of lymphoid predominance develops into diffuse large B-cell non-Hodgkin lymphoma. This variant of lymphogranulomatosis is characterized by a favorable prognosis, although it may have a chronic relapsing course that lasts for many years, similar to low-grade non-Hodgkin lymphomas.

Symptoms and signs of lymphogranulomatosis

Generalized lymphadenopathy is possible.

In a later stage - symptoms of damage to the liver, lungs, and bone marrow.

General symptoms (“B” symptoms):

• fever;
• night sweats;
• weight loss. Other systemic symptoms:
• pain in the lymph nodes when drinking alcohol.

Characterized by an extreme variety of symptoms. The disease begins with an enlargement of the lymph nodes of the neck (initially on the right), then the process moves to the other side and nodes of other areas. The size of lymph nodes can vary from the size of a pea, bean to a man's fist. The nodes are soldered together into conglomerates, but are not soldered to the skin, do not open and do not suppurate.

Fever always accompanies lymphogranulomatosis and in the early stages may be its only sign. The temperature reaction is varied, but in most cases it is characterized by a wave-like character. Skin itching may be localized over the area of ​​enlarged lymph nodes or generalized, painful.

Laboratory tests: maybe hypochromic anemia and neutrophilic leukocytosis; the number of lymphocytes (absolute and relative) is reduced; Eosinophilia and thrombocytopenia may be observed; ESR increases moderately, but in the final stage reaches mm/h.

Stages of lymphogranulomatosis

Lymphogranulomatosis is characterized by the spread of tumor cells from the affected lymph node to neighboring nodes. Therefore, when treating patients, they are guided by the Ann Arbor classification. The stage of the disease is currently determined by the results of a CT scan of the neck. chest, abdomen and pelvis rather than laparotomy. Bone marrow involvement is rarely detected at the time of diagnosis, but it is more likely when the lesion is localized below the level of the diaphragm, therefore, in such cases, bone marrow terpanobiopsy is also performed to clarify the stage of the tumor process. The identification of other prognostic factors has led to the fact that when choosing treatment tactics, they rarely focus only on the anatomical stage of the disease.

• I - damage to one or one group of nodes;
• II - damage to several groups of lymph nodes on one side of the diaphragm;
• III - damage to the lymph nodes on both sides of the diaphragm, spleen;
• IV - involvement of extranodal zones (with the exception of tumor growth “along its length”).

Each stage requires clarification by adding one of the following letter indices: A - general symptoms absent; B - there are general symptoms; E - there are symptoms of damage to various organs.

Research in recent years has made it possible to identify signs of the disease that have prognostic significance. For the early stage of the disease (stages I and IIA), prognostic groups have been formulated based on the histological variant of the disease, the age and gender of the patient, its symptoms, the number of affected anatomical areas and damage to the mediastinal lymph nodes.

For the advanced stage of the disease (stage IIB-IV), seven prognostic factors were identified based on an analysis of 5000 cases of the disease.

In the absence of these factors, the 5% disease-free survival rate is 84%. The presence of each of these factors reduces expected survival by approximately 7%. If three or more factors are present, the prognosis is considered unfavorable.

Clinical forms of lymphogranulomatosis

There are transient forms lasting several months, forms lasting 2-3 years, and lasting 5-6 years or more.

According to the degree of prevalence of the process, in accordance with the WHO classification, they are distinguished:

1. local form with damage to one group of lymph nodes;
2. regional form with damage to no more than two groups of lymph nodes in one area (above or below the Diaphragm);
3. generalized form with damage to many lymph nodes above and below the diaphragm;
4. disseminated form with damage not only to the lymph nodes, but also to internal organs, as well as bones, central nervous system, serous and meninges.

The prognosis is unfavorable. Average duration life is 3-4 years, some patients live 6-8 years or more. Long-term and persistent remissions are possible.

Diagnosis of lymphogranulomatosis

In the diagnosis of mediastinal, pulmonary, bone, abdominal forms in the presence of typical general clinical symptoms, radiological research methods - radiography, lymphadenoangiography - become of great and often decisive importance.

The diagnosis of lymphogranulomatosis is based on a biopsy of the affected lymph nodes and other tissues with staining of preparations using traditional and immunohistochemical methods. The drugs should be examined by an experienced hematologist. Fine needle aspiration biopsy can also be used, but is not sufficient to make a diagnosis on its own.

The diagnosis is based on the triad: swollen lymph nodes, fever and itching. It is considered reliable upon histological confirmation - the detection in a biopsy of a lymph node of a specific granuloma containing giant Sternberg cells (mm in diameter).

Treatment of lymphogranulomatosis

It consists of wide-field or total X-ray irradiation of the lymph nodes and polychemotherapy with a combination of cytostatics.

In some cases, in the presence of monolocal (or monoorgan) lymphogranulomatosis, radical surgery- removal of individual packages of lymph nodes, resection of the stomach, intestines, etc., giving long-term clinical remission - up to 10-15 years or more.

Of the active therapeutic agents used for lymphogranulomatosis, X-ray therapy is most widespread. Local deep therapy of the affected lymph nodes is carried out. The best results are obtained by telegammatherapy (“cobalt gun”), applied topically in single doses of 200 rads (for a course of treatment 4000-8000 rads). For prophylactic purposes, not only the lesion is irradiated, but also neighboring areas, and in some cases, the spleen.

Severe leukopenia or thrombocytopenia is a contraindication to further radiotherapy.

For generalized lymphogranulomatosis, treatment with chemotherapeutic agents is indicated.

One of the most effective cytostatic drugs that have proven themselves in the treatment of generalized forms of lymphogranulomatosis are the pink vinca alkaloids vinblastine (Hungarian) and vincristine (American). The drugs are administered only intravenously, in a 5% glucose solution or distilled water. Vinblastine is used in a dose of 5-10 mg initially, if well tolerated, every other day, then after 3-5 infusions, in the same doses once every 5-10 days. Vincristine, due to its more pronounced toxic properties, is administered in half doses - 2.5-5 mg.

With the development of deep leukopenia (2000 and below), treatment is temporarily interrupted.

In order to ensure the maximum concentration of the chemotherapy drug in the lesion with minimal general toxic effect, an endolymphatic method of administering cytostatics in an increased dose is proposed - 150-200 mg of dipine or degranol with preliminary and subsequent infusion into lymphatic vessels 5-6 ml of 1% novocaine solution (to prevent pain at the injection site).

IN last years Attempts are being made to “radical” chemotherapy for lymphogranulomatosis through polychemotherapy - the simultaneous use of several chemotherapy drugs.

The most commonly accepted combinations of cytostatics are:

1. vinblastine + bruneomycin;
2. vincristine + cyclophosphamide + methotrexate 2+ prednisolone;
3. vin-blastine + cyclophosphamide + bruneomycin + prednisolone.

A promising method aimed at complete “eradication” (eradication) of the disease is the proposal to use cytostatics in maximum (many times higher than normal) doses, causing aplasia of hematopoiesis, followed by autotransfusion in advance (before the start of treatment) of harvested autologous (own) bone marrow stored in during the course of treatment in the refrigerator at a temperature of -70°.

In order to prevent drug-induced cytopenia (leuko-thrombocytopenia) or its rapid elimination, treatment with cytostatics is carried out under the protection of hemotherapeutic (transfusion of red blood cells, leukocytes, platelets) and hormonal (prednisolone) agents.

Treatment of patients with lymphogranulomatosis should be carried out in specialized oncohematology clinics. All patients with lymphogranulomatosis, as well as others systemic diseases blood, are subject to dispensary observation in oncology and hematology dispensaries.

Since lymphogranulomatosis mainly affects young people, the problem of side effects of therapy, given the significant increase in the life expectancy of patients, becomes very relevant. An analysis of late side effects of treatment for lymphogranulomatosis showed that over a 30-year period of observation of patients with lymphogranulomatosis, 2 times more patients died from tumors that developed de novo than from relapses of the disease itself. Analysis of long-term complications of radiation therapy, especially after irradiation of the mediastinum ( lung cancer and breast, pulmonary fibrosis, coronary heart disease), served as the basis for changing the approach to high-dose radiation therapy. Irradiation of the mantle zone in women under the age of 20 leads to the development of breast cancer by the age of 50 in every third patient, so such radiation therapy is no longer used. Chemotherapy with alkylating drugs promotes the development of secondary myelodysplasia, acute myeloid leukemia and non-Hodgkin's lymphoma, as well as infertility, which makes it necessary to take this circumstance into account when planning treatment. The doctor’s goal when prescribing treatment is to achieve a cure for as many patients as possible and at the same time reduce the incidence of adverse long-term consequences, especially when treating patients at an early stage of lymphogranulomatosis.

Early stage (IA and NA)

In most patients with the early stage of lymphogranulomatosis, supraphrenic lymph nodes are affected at the time of diagnosis. Care of these patients should be carried out taking into account prognostic factors that predict the likelihood of damage to the subdiaphragmatic lymph nodes, if there are no obvious signs of their involvement in the tumor process during the usual staging of the disease.

In patients with a favorable form of the disease, in particular nodular sclerosis or a nodular variant of lymphoid predominance, occurring with damage to the upper cervical lymph nodes and low ESR, the likelihood of damage to the subphrenic lymph nodes is very low. They can only be treated by irradiating the affected group of lymph nodes. However, most patients with a favorable prognosis and early stage lymphogranulomatosis outside clinical trials should also be given ABVD chemotherapy followed by irradiation of the affected group of lymph nodes. The relapse rate after such treatment was the same as after irradiation of the mantle zone or total irradiation of all lymph nodes. Even a shorter 4-week chemotherapy according to the VAPEC-B regimen with irradiation of the affected group of lymph nodes gives very good results.

Recently, to detect an active tumor process in residual enlarged lymph nodes, PET with Tordex-glucose labeled with 18 F has been successfully used. This research method, used after completion of standard therapy, has a high predictive value of positive (87.5%) and negative (94.4%) results regarding relapses in patients with early stage lymphogranulomatosis. It remains to be seen whether PET can be used to identify patients who can avoid local radiation after chemotherapy without increasing the risk of relapse. An NCRI study currently underway in the United Kingdom will answer this question. Patients in this study receive three courses of ABVO chemotherapy followed by a PET scan. If the PET scan reveals an active process, another course of ABVD is given, followed by irradiation of the affected lymph nodes. Patients with negative result studies are randomly assigned to one of two groups, one receiving local radiotherapy and the other not.

Patients with an early stage of the disease, but an unfavorable prognosis, are treated as for the advanced stage of lymphogranulomatosis according to the generally accepted scheme.

Advanced stage of the disease

ABVD and other doxorubicin-containing chemotherapy regimens have become widely accepted as standard therapy since the completion of the CALGB study. This study compared the effectiveness of three regimens: MOPP (mustine, vincristine, procarbazine, prednisone), alternating MOPP and ABVD, and ABVD alone.

Recently, short polychemotherapy Stanford V (mustine, doxorubicin, vinblastine, prednisolone, vincristine, bleomycin, etoposide) and high-dose polychemotherapy BEACORR have been developed. These treatment regimens are combined with local radiation therapy to the affected lymph nodes. By preliminary results, such treatment is effective in most patients and provides high disease-free survival. Thus, when treated according to the Stanford scheme, the 3-year overall and relapse-free survival rate in patients in the advanced stage of lymphogranulomatosis was 96% and 87%, respectively. Very impressive results were obtained with treatment according to the BEACOPP regimen; they turned out to be better than when alternating the SOPP and ABV regimens. Attempts to further increase the dose of drugs in the escalated BEACORR regimen made it possible to enhance the antitumor effect of therapy, but at the same time, treatment-related cases of the development of myelodysplastic syndrome and acute myeloid leukemia increased significantly. Mixed therapy of four courses of BEACORR according to the usual regimen and four courses according to the regimen with an increased dose of drugs allowed us to obtain very high survival rates and reduce the incidence of myelodysplastic syndrome. In a recent United Kingdom study, alternating ChlVPP and PABLOE or hybrid ChlVPP/EVA regimens were not superior to ABVD. Until the benefit of BEACOPP therapy is finally proven, the ABVD regimen will remain the most widely used in the treatment of patients with lymphogranulomatosis.

Further treatment

If a relapse occurs after radiation therapy in patients with early stage lymphogranulomatosis, subsequent chemotherapy is quite effective (80-90% of patients can significantly prolong life without relapse of the disease). If relapses occur after first-line chemotherapy, second-line chemotherapy can be successfully prescribed, especially if the previous remission lasted more than 12 months. However, it is possible to significantly increase life expectancy when prescribing chemotherapy in usual doses only in 20-25% of patients.

High-dose chemotherapy followed by autologous stem cell transplantation can significantly improve progression-free survival (DFS) and is currently considered the standard treatment for patients with recurrent lymphogranulomatosis. It allows you to significantly prolong disease-free survival in 40-50% of patients.

Prospects

Refusal to use radiation therapy at an early stage of the disease.

The use of antibodies to the CD30 antigen of Reed-Sternberg cells; Phase II clinical trials have shown some effect with these antibodies.

The use of anti-CO20 antibodies, including those labeled for the treatment of the nodular variant with lymphoid predominance.

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Lymphogranulomatosis

A malignant change in lymphoid tissue with granulomas and Berezovsky-Sternberg cells (a morphologically distinct group of cells, unlike anything else) is called “lymphogranulomatosis” (cancer of the lymphatic system). The main symptom of the disease is a significant increase in lymph nodes. Another name is Hodgkin's disease.

What is lymphogranulomatosis?

Thomas Hodgkin is a British doctor who first suggested that enlarged lymph nodes are not a consequence of an inflammatory process or metastases of another tumor, but that they are an independent disease. The tumor consists of large multinucleated cells that are localized in the affected lymph nodes.

The disease affects a young group of the population: children, adolescents, adults of childbearing age. The peak occurs between the ages of 14 and 35 years. The development of the disease is also observed after 50 years. Men get sick 40% more often. The incidence of the disease is stable at 25 cases per million population annually.

Causes

The exact cause that contributes to the development of the disease is currently unknown. Hodgkin's lymphoma is not transmitted between family members. High risk(up to 99%) in identical twins. The appearance of lymphogranulomatosis (B-cell lymphoma) is presumably associated with the Epstein-Barr virus (herpes virus type four). This association is due to the fact that the virus renews itself (replicates) in B lymphocytes and activates their reproduction by division.

First signs and mature symptoms of the disease

The disease begins with enlargement of the submandibular and cervical lymph nodes. On initial stages the node is dense, not very painful, mobile, and feels like a cherry fruit, which can grow to the size of an apple or more. There is no special gradation regarding the volume of the enlarged node. Its value is individual in each individual case.

Later, the subclavian lymph nodes, intrathoracic (mediastinal), and less often inguinal lymph nodes are involved in the pathological process.

Late signs are a large massive tumor in the retroperitoneum, mediastinum, which can lead to compression of the trachea, superior vena cava, and patients experience shortness of breath. The spleen enlarges significantly, sometimes its size is so large that the organ occupies half of the abdominal cavity. The patient's condition is serious, there is constant temperature body 37.5°, heavy sweats at night, sudden weight loss (more than 10% of total mass body), incessant skin itching.

Stages of distribution of the disease in the body:

1. Single lymph nodes.
2. Different groups of nodes on one side of the diaphragm.
3. Groups of nodes on both sides of the diaphragm (advanced cancer).
4. Damage to organs and tissues.

How is Hodgkin's disease diagnosed?

The examination algorithm depends on the location of the nodes, their consistency and density, as well as the condition of the surrounding tissues.

The diagnosis is made only by histology results. To do this, a biopsy of the affected lymph node is performed. A morphologist evaluates the result and, based on his conclusion, a final diagnosis is established.

A similar increase in nodes is characteristic of diseases such as tuberculosis, sarcoidosis (granular lesions of organs, most often the lungs), syphilis, collagenosis (pathology connective tissue). Therefore, the specificity of the nodes is determined only by morphological examination of tissue samples.

Other diagnostic methods:

1. palpation of nodes, spleen, liver;
2. examination of the tonsils;
3. examination of bone marrow from the iliac wing;
4. CT ( CT scan) chest and abdominal cavity;
5. clinical and biochemical blood test with analysis of liver and kidney function;
6. PET ─ positron emission tomography with labeled glucose;
7. ECG and ECHO cardiography ─ is performed if treatment with chemotherapy drugs with cardiotoxicity is expected;
8. endoscopic examination of the gastrointestinal tract ─ if the upcoming therapy can lead to ulcerative changes in the mucous membrane of the digestive tract.

Treatment of lymphogranulomatosis. Scientific medicine and folk remedies

Treatment of lymphogranulomatosis is carried out strictly according to international protocols. The volume of treatment corresponds to the volume of the lesion, so the choice of therapy is always individual.

The first stage is polychemotherapy, where tactics are important ─ maximum dose and minimum intervals. In some cases (not always), radiation therapy is used to remove residual effects. The number of courses, their intensity, and areas for radiation therapy are determined for each patient separately.

Chemotherapy for lymphogranulomatosis has a destructive effect on male germ cells. And since mostly young men are affected, before treatment everyone must donate their seminal fluid to a special bank for cryopreservation (freezing) and storage. This is the only opportunity to have children in the future. For women, the situation is simpler; medicine has learned to protect the ovaries during chemotherapy by blocking them with hormonal drugs.

Drug treatment is often combined with traditional medicine. The use of properly selected herbs helps:

• slowing down or stopping tumor growth ─ chaga mushroom, tansy, calendula, alfalfa, ginseng, St. John's wort;
• cleansing the body of chemotherapy drugs ─ yarrow, dill seeds, sage, plantain, sweet clover;
• restoration of hematopoietic function ─ red beet juice, nettle;
• increasing the body's resistance ─ raspberries, propolis tincture, aloe syrup.

Infusions, decoctions, juices, and herbal mixtures are prepared from these herbs.

Prognosis and survival

The disease can cause relapses, with 70% of them occurring 2-3 years after treatment, and complications.

• damage to the gastrointestinal mucosa;
• infections of the upper respiratory tract and pneumonia;
• toxic liver damage;
• hepatitis B and C.

• secondary tumors;
• cardiovascular diseases;
• osteoporosis;
• thyroid dysfunction;
• infertility.

With the correct stage of the disease and adequate treatment, the success rate of recovery is 90% or more. In children, the recovery process occurs faster, and relapses are rare. The main thing to remember is that effective treatment And early treatment─ these are synonyms.

Adults in 85-90% of cases at stages 1 ─ 4 have long survival without manifestation of the disease. 5-10% are patients of the resistant group who do not respond to treatment. 5-10% ─ Hodgkin's disease with relapses. Only through the joint efforts of doctors, the patient and his family, using modern treatment methods, can lymphogranulomatosis be overcome.

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The information on the site is presented for informational purposes only! It is not recommended to use the described methods and recipes for treating cancer on your own and without consulting a doctor!