Lymphogranulomatosis (Hodgkin's disease). Hodgkin's lymphoma disease Mixed cell Hodgkin's lymphoma


One of the most dangerous diseases is Hodgkin's lymphoma or lymphogranulomatosis. This form of lymphoma has a number of features. The disease gradually affects all lymph nodes. Its character is predictable, and treatment is a little different than for other malignant diseases.

Lymphoma or cancer of the lymphatic system, may be Hodgkin's or non-Hodgkin's. Hodgkin's lymphoma most often affects regional lymph nodes. The diagnosis is made on the basis of a biopsy (analysis of biological material taken from lymph nodes). With the Hodgkin type of lymphoma, doctors detect pathological specific Reed-Sternberg cells in the lymph nodes.

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Causes and symptoms of Hodgkin's lymphoma

It is impossible to name the exact cause of this disease. Experts conduct scientific research trying to establish the causes of the disease.

The appearance of a painful swelling is the initial sign of lymphoma.

The initial sign is the appearance of a painful swelling. It can occur in the armpit, groin or neck.

Other symptoms:

  • loss of appetite, poor health, general weakness, weight loss;
  • increased temperature, increased sweating at night;
  • shortness of breath, cough, abdominal pain (in children);
  • when palpating the abdomen, a neoplasm can be identified (in children);
  • painful and swollen lymph nodes (pain appears after drinking alcohol).

Sometimes there are no individual symptoms of the disease. In some cases, it is confused with a cold. People turn to a specialist with signs of advanced acute respiratory infections, but they are diagnosed with lymphoma.

Thus, if a person has had cold symptoms for a long time, this should raise some suspicions. But we must remember that these symptoms are similar to many diseases that are not malignant in nature.

Hodgkin's lymphoma can have two types:

  • classical,
  • nodular.

Hodgkin's lymphoma - PHOTO

The photo shows Hodgkin's lymphoma - swollen lymph nodes in the neck:

Diagnostics

Hodgkin's lymphoma is not spread from person to person. It is not genetic in nature and is not inherited from parents.

During the diagnostic process, the specialist conducts a general examination, collects anamnesis and prescribes radiography. This allows you to assess the general condition of the patient.

The doctor gives a final answer about the presence or absence of lymphoma only after receiving the results of the biopsy. This operation is performed under local anesthesia. The material for research is taken using a miniature needle, which is inserted into the swollen lymph node. In this case, monitoring is carried out using computed tomography.

The biopsy may include the following procedures:

  • lymph node removal;
  • removal of a specific area of ​​a lymph node;
  • taking a piece of tissue from a lymph node using a wide needle (puncture biopsy);
  • taking tissue from a lymph node using a thin needle (fine needle aspiration biopsy).

If a patient is diagnosed with Hodgkin lymphoma, then additional examinations are prescribed.

Doctors distinguish 4 histological types of this disease:

  • lymphoid predominance,
  • nodular sclerosis,
  • nodular with lymphoid predominance,
  • mixed cell version,
  • lymphoid depletion.

There is a relationship between the type of disease and its course. The most common diagnosis is nodular sclerosis of Hodgkin lymphoma. Typically this form of the disease affects women. In this case, doctors detect enlarged cervical lymph nodes.

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How is Hodgkin's lymphoma diagnosed in Israel?

Many patients prefer to undergo examination in medical centers in Israel. The following diagnostic tests are carried out there:

  • blood tests (laboratory tests);
  • ultrasonography;
  • chest x-ray;
  • computed tomography of the whole body;
  • bone marrow biopsy;
  • Magnetic resonance imaging.

Stages of Hodgkin's lymphoma

  • Stage 1. The malignant process affected only one group of lymph nodes. For example, on one side of the groin or on one side of the neck.
  • Stage 2. 2 or more groups of lymph nodes are affected, but they are all located on the same side of the diaphragm (above or below it).
  • Stage 3. At stage 3 of Hodgkin's lymphoma, the malignant process has affected the lymph nodes located on both sides of the diaphragm.
  • Stage 4. Cancer cells are found in lymph nodes, bones and internal organs.

There is a classification of lymphoma based on symptoms. In this case, doctors use letter designations:

  • Category A- the following symptoms are present: fever at night, sweating and weight loss.
  • Category B- the patient does not have the above symptoms.

Hodgkin's lymphoma in children

There are two types of Hodgkin's lymphoma in children:

  • classic type,
  • nodular type (lymphocytes predominate).

The risk of developing the disease increases if the Epstein-Barr virus is present in the child's body. Boys aged 5 to 14 years are more likely to be diagnosed with lymphoma. If a child has a sister or brother with this diagnosis, the risk also increases.

Signs of the disease in children are the same as in adults.

Treatment of Hodgkin's lymphoma

Treatment depends on the stage of development and type of lymphoma. Treatment tactics are also influenced by the patient’s general well-being and concomitant diseases.

The goal of treatment is complete remission (disappearance of symptoms and signs of the disease). If complete remission cannot be achieved, then doctors talk about partial remission. In this case, the tumor shrinks and stops infecting new tissues of the body.

The goal of treatment is complete remission.

Treatment for Hodgkin lymphoma includes:

  • drug methods (immunotherapy and chemotherapy),
  • radiotherapy (irradiation).

Sometimes surgical treatments are also used. In the early stages of Hodgkin lymphoma, chemotherapy and radiotherapy are used. In later stages, chemotherapy, radiotherapy and immunotherapy are used.

  • Radiotherapy allows you to destroy malignant cells in lymph nodes or internal organs. Radiation is given to all areas of the body where cancer cells are thought to have spread.
  • Chemotherapy for Hodgkin's lymphoma involves taking special medications that destroy malignant cells. Today doctors prescribe pills and intravenous injections. To increase the chances of recovery, several different medications are used at once. The combination of drugs is compiled by a specialist based on the individual characteristics of the patient.
  • Immunotherapy aimed at supporting the human immune system. At home, you can use traditional methods of treating cancer.

We list the most effective methods of traditional healers:

  1. Syrup obtained from aloe. It is a general tonic natural remedy.
  2. A decoction of viburnum berries and branches is drunk for malignant neoplasms.
  3. Infusion of calendula flowers. It is enough to pour 2 tablespoons of raw materials with boiling water (2 cups). After half an hour, the infusion can be taken. You can drink it instead of tea.
  4. Chaga infusion. This is a birch mushroom that is useful for cancer. If you take it in the initial stages, you can stop the growth of the tumor and reduce pain. The infusion can be taken about three times a day, a tablespoon (before meals).

Relapse

Reactivation of malignant cells occurs in many patients. Therefore, after completing the treatment course, patients should regularly come for examination by the treating specialist. This makes it possible to detect a relapse of Hodgkin lymphoma in time and take action.

Inspection must be done every 2 months.
A CT scan should be performed every 4 months.

Diet for Hodgkin's lymphoma

A special diet will help alleviate the condition.

  • The patient should eat dairy products. Fermented milk products are especially beneficial.
  • It is not recommended to eat salty, fatty, sweet, fried foods.
  • Fast food, alcohol and carbonated drinks are prohibited.
  • You should include fresh fruits and vegetables in your diet.

The following products are useful: soups, buckwheat and oatmeal, beets, carrots, cheese, garlic, etc.

Treatment method for Hodgkin's lymphoma in Israel

Traditional methods of treating this disease are radiotherapy and chemotherapy. They are used separately and together.

Traditional treatments for lymphoma are radiotherapy and chemotherapy.

The treatment plan is drawn up by a team of highly qualified specialists. They take into account many factors: the stage of lymphoma, the location of painful nodes, their degree of enlargement, the general health of the patient, concomitant diseases, etc.

If treatment occurs in the early stages of lymphoma, radiotherapy is used. This method is often sufficient. But most often chemotherapy is added to radiotherapy even in the early stages. Late stages of Hodgkin's lymphoma are treated with high-dose chemotherapy.

Nodular sclerosis is a histological type of lymphogranulomatosis, characterized by dense growth of connective tissue, divided into a mass of irregularly shaped cells and lobules. They contain expanded lymphoid matter with a huge number of Berezovsky-Sternberg cells. The disease begins with an increase in nodes. This pathology is one of the variants of classic Hodgkin lymphoma.

Hodgkin's disease is considered a serious illness that affects the lymphatic system. The disease can form in any organ that has lymphoid tissue (tonsils, spleen, adenoids, etc.).

Nodular sclerosis: symptoms

A person may have Hodgkin's lymphoma if they have symptoms such as:

  • weight loss;
  • enlarged lymph nodes (often in the neck);
  • loss of appetite;
  • dyspnea;
  • night sweats or fever;
  • chest pain;
  • enlarged liver (5% of patients) or spleen (30% of patients);
  • heaviness or pain in the abdomen (in children);
  • itchy skin (only in 1/3 of people suffering from the disease);
  • difficulty breathing;
  • cough.

Causes

Lymphogranulomatosis can be contracted at any age, but more often it is observed in young men aged 16 to 30 years or in elderly people over 50 years of age. Children under 5 years old practically do not get sick. What exactly provokes this disease is still unknown. However, there is an assumption that the source is viruses. It is believed that the onset of this ailment can be caused by:

  • immunodeficiency states;
  • infectious mononucleosis (caused by the Epstein-Barr virus).

Nodular sclerosis of Hodgkin's lymphoma can resolve instantly, last from 3 to 6 months, or last for 20 years.

What stages does the disease have?

The grades of Hodgin lymphoma are determined based on the results of laboratory tests and on the basis of the following indicators:

  • the number of people affected and their location;
  • the presence of these nodes in different areas of the diaphragm;
  • tumors in other organs (for example, in the liver or spleen).

First stage. In this case, only one lymph node or lymphoid organ is affected (spleen, Pirogov-Walder ring).

Second stage. Here the lymph nodes on both sides of the chest, the diaphragm and the lymphoid organs are usually affected.

Third stage. This stage of Hodgkin lymphoma is almost no different from the second stage. However, it has two types of nodular sclerosis of the third stage:

  • in the first case, organs located below the diaphragm are affected (abdominal lymph nodes, spleen);
  • in addition to the areas listed in the first type, other areas with lymph nodes located near the diaphragm are also affected.

Fourth stage. Not only the nodes are affected, but also non-lymphoid organs - bone marrow, liver, bones, lungs and skin.

Designations of the degrees of Hodgkin's lymphoma

The indicator of the severity of the clinical situation and the painful course of other tissues and organs is marked with letters.

A - there are no severe general manifestations of the disease.

B - the presence of one or more symptoms (unreasonable increased temperature, sweating at night, rapid weight loss).

E - lesions spread to tissues and organs located near the affected lymph nodes.

S - there is damage to the spleen.

X - there is a serious tumor of enormous size.

Histological types of the disease

Regarding the cellular structure of lymphogranulomatosis, there are 4 forms of illness.


Diagnostics

The diagnosis of lymphoma is determined only by histological examination of the lymph nodes and is considered proven only when, as a result of this study, special multinucleated Sternberg cells were discovered. In severe cases, immunophenotyping is needed. Cytological analysis of a lymph node or kidney puncture is usually not enough to confirm type 1 nodular sclerosis. What needs to be done to establish a diagnosis of the disease:

  • general and biochemical blood test;
  • X-ray of the lungs (mandatory in lateral and direct projection);
  • lymph node biopsy;
  • ultrasound examination of all types of peripheral and intra-abdominal thyroid glands, liver and spleen;
  • computed tomography of the mediastinum to eliminate lymph nodes that are not visible on traditional radiography;
  • trephine biopsy to exclude bone marrow damage;
  • bone scans and x-rays.

Therapy

Contains radiation treatment, surgery and chemotherapy. The choice of method is determined by the stage of the disease and the presence of positive or negative prognostic reasons. Favorable factors include:

  • nodular sclerosis and lymphohistiocytic type revealed during histological examination;
  • age less than 40 years;
  • volumes of lymph nodes that do not exceed 6 cm in diameter;
  • lack of general manifestations of biological effectiveness (development of biochemical blood parameters);
  • presence of no more than 3 lesion locations.

If at least one of these reasons is absent, then the patient is included in the group with an unfavorable prognosis.

Radiation treatment

Total radiation therapy as an individual method is used for patients with stages IA and IIA, confirmed by laparotomy, and having good prognostic factors. It is done with free fields with irradiation of any types of affected areas as well as lymph outflow passages.

The total absorbed portion in the metastases of the lesion is 40-45 g in 4-6 weeks, in places of preventive irradiation - 30-40 g in 1-4 weeks. Also with wide-field, methods of multi-field irradiation of some lesions are used to prevent nodular sclerosis ns1.

Radiation treatment can cause complications such as subcutaneous tissue fibrosis, radiation pulmonitis and pericarditis. Deterioration appears at different periods - from 3 months to 5 years after therapy. Their complexity depends on the dose used.

Operations

Surgical treatment is rarely used separately; it is usually part of a complex therapy. Splenectomy is performed, as well as operations on the trachea, esophagus, stomach and other organs (if there is a risk of asphyxia or food passage disorder). A pregnancy discovered during ongoing lymphogranulomatosis must be terminated.

Chemotherapy

This type is used as one of the components of complex treatment. To treat nodular sclerosis, different drugs are used:

  • alkaloids (“Vinblastine” or “Rosevin”, “Etoposide” or “Vincristine”, “Oncovin”);
  • alkating mixtures (“Mustargen”, “Cyclophosphamide” or “Embiquin”, “Nitrosomethylurea” or “Chlorobutin”);
  • synthetic agents (“Natulan” or “Procarbazine”, “Dacarbazine” or “Imidazole-Carboxamide”);
  • antitumor antibiotics (“Bleomycin”, “Adriablastin”).

Monochemotherapy

It is used only in special cases for illustrative purposes. As a rule, therapy with several medications with different mechanisms of action is prescribed (polychemotherapy). At the fourth stage in patients with diffuse damage to the liver or bone marrow, this type of treatment is the only way - this is classical Hodgkin lymphoma. Nodular sclerosis is treated according to the following schemes:

  • ABVD (“Bleomycin”, “Dacarbazine”, “Adriablastine”, “Vinblastine”);
  • MOPP (“Oncovin”, “Prednisolone”, “Mustargen”, “Procarbazine”);
  • CVPP (Vinblastine, Prednisolone, Cyclophosphamide, Procarbazine).

Therapy is carried out in short-term (2, 7, 14 days) courses with two-week breaks. The number of cycles varies depending on the size of the initial lesion and responsiveness to treatment. Typically, complete remission is achieved with the prescription of 2-6 courses. After this, it is recommended to perform 2 more cycles of therapy. If the result was partial remission, then the treatment regimen changes and the number of courses increases.

Taking medications is accompanied by blood pressure, alopecia, and dyspeptic symptoms, which go away at the end of treatment. Nodular sclerosis also leads to such late complications as infertility, leukemia and other malignancies (secondary tumors).

Forecast

It is determined by the peculiarities of the course of lymphogranulomatosis, the clinical stage of the disease, the age of the patient, histological type and others. With a sharp and subacute process of the disease, the prognosis is not good: patients usually die between 1-3 months and up to 1 year. But with chronic lymphogranulomatosis, the prognosis is conditionally positive. The disease can last a very long time, up to 15 years (in some cases much longer).

In 40% of all infected people, especially with stages 1 and 2, as well as favorable prognostic reasons, there are no relapses for 10 years or more. The ability to work is not impaired as a result of prolonged remissions.

Prevention

As a rule, it is aimed at preventing relapses. Patients with lymphogranulomatosis are subject to a clinical examination by an oncologist. During the study, which must be carried out every six months for the first 3 years, and then once a year, it is necessary to focus on biological indicators of effectiveness, which are often the initial signs of relapse (increased levels of fibrinogen and globulins, increased POPs). For patients with lymphogranulomatosis, thermal physiotherapeutic procedures, overheating and direct insolation are harmful. An increase in the number of relapses due to pregnancy has been established.

Now many people probably know that Hodgkin lymphoma is a variant of nodular sclerosis, which is a very unpleasant and difficult to treat disease.

They become uncontrollable, begin to divide, become multi-core and very aggressive, especially in the last stages. The cells devour tissues and lymph nodes in the human body, thereby destroying all filtering organs.

The nodular type of lymphoma exists in varieties of Hodgkin's disease. This disease has Reed Stenberg cells, which is a type of classic lymphogranulomatosis disease.

At the initial stage of sclerosis of this type, patients do not show any symptoms. At first, the disease is quiet and painless. Only the lymph nodes gradually enlarge.

But the fact that Hodgkin's disease exists has already been verified more than once. It has also been verified that this type of lymphoma develops like the nodular type due to viral infections.

In addition, to this day no one knows the true causes of this disease.

Symptoms

The first manifestation of this type of sclerosis disease will be enlarged lymph nodes. Often the process begins in the cervical-supraclavicular areas, where the lymph nodes are located.

Inflammation of the lymph nodes in the neck

Usually the process starts on the right. In this case, the patient does not complain about his health, nothing bothers him. Some patients come to the clinic only concerned about enlarged nodes and nothing more.

Then the pathology gradually gains momentum and suddenly appears in the organs. In this case, the person suffers from intoxication. He begins to feel very nauseous. If a specific organ is affected by a nodular type of disease, then doctors, through examination, determine its clinical picture.

In nodular disease, the lymph nodes are mobile and not adherent to the skin. They become densely elastic, the nodes increase gradually. In some cases, they merge so tightly that large conglomerates are formed.

During the course of a disease such as sclerosis, you should not drink alcoholic beverages, as this can lead to very severe pain in the lymph nodes. Lymph nodes expand with this type of disease, affecting the spleen and liver. Anemia may occur and will progress over time.

Symptoms of lymphoma appear in the form of:

  • profuse night sweats;
  • weakness, fatigue;
  • shortness of breath, cough;
  • enlarged lymph nodes;
  • itching, which can occur anywhere.

Diagnosis of nodular sclerosis

In patients with painless lymphadenopathy or mediastinal adenopathy, it is detected during routine X-ray examination. In order to correctly diagnose a patient, it is necessary to identify the stage of lymphoma.

Patients undergo liver and kidney tests. The initial stage shows damage to one group of lymph nodes. At the second stage, lymphoma can affect the lymph nodes and another organ.

Hodgkin cell in a cytological specimen

At the third stage, nodular sclerosis is detected. In this case, lymphoma affects the lymph nodes on both sides of the diaphragm. The fourth stage is characterized by a classic pathological process, which involves many groups of lymph nodes, lungs, liver, and skeletal system. The bones are involved in the process asymptomatically.

In this case, only:

  • the occurrence of vetebral osteoblastic lesions (elephant vertebrae);
  • the appearance of osteolytic lesions and compression fractures, and pain associated with such pathology.

Local compression of tumor masses include:

  1. jaundice due to intrahepatic or extrahepatic obstruction of the bile ducts;
  2. swelling of the legs due to obstruction of the lymph ducts in the groin and pelvis;
  3. shortness of breath, hoarse breathing, tracheobronchial compression;
  4. laryngeal paralysis due to enlarged nodes;
  5. neuralgia due to the nerve root;
  6. pulmonary abscess, resulting in a simulation of lobar consolidation or bronchopneumonia.

There is no easy way to identify this disease. This work is a labor-intensive and time-consuming process.

Various diagnostic methods and techniques are used:

  • First, doctors practice taking a medical history. To do this, they invite the patient to the clinic and talk with him. Externally examine the patient. Palpation is performed. The patient is sent for an x-ray to clear the lungs;
  • fluoroscopy
  • An ultrasound is performed to get acquainted with the condition of the internal organs. They offer to undergo computed tomography, magnetic resonance imaging;
  • they send you to do an endoscopy of the larynx and bronchi;
  • a node with a tumor is taken for biopsy, then studies related to histology and immunology are carried out;
  • CSF (cerebrospinal fluid) is examined to identify cancer cells. To study the cerebrospinal fluid, a lumbar puncture is performed;
  • The bone marrow is biopsied. Due to the fact that the development of leukemia, lymphogranulomatosis, reticulosarcomatosis may occur;
  • They take blood for a detailed analysis. The most important analysis is considered to be a biopsy sample. Cells or tissues are taken from the body intravitally and examined. Thanks to it, the stage of the disease and its development are determined;
  • General blood tests will assess how many white blood cells, platelets, red blood cells and hemoglobin are in the blood. This is important because if there is a disease, the picture of qualitative and quantitative indicators will change significantly;
  • biochemical tests will help determine the state of functioning of body systems. Here they look at the functioning of the liver, kidneys, metabolism, inflammatory processes;
  • immunological tests are also blood tests, they are collected in order to verify the presence of lymphoma. These types of tests are necessary to determine the stage corresponding to this disease.

Immunity is directly related to the lymph systems; as a result of the disease, a sharp decrease in the body’s defenses occurs. As a result, the body simply stops fighting.

A peripheral blood test can detect changes in blood due to malignant tumors:

  1. anemia - anemia;
  2. leukocytosis, with an increased number of leukocytes;
  3. increased ESR indicators of the rate at which red blood cells settle;
  4. The blood may change due to a reduced concentration of lymphocytes, this process is otherwise called lymphopenia.

Usually the patient is sent for an x-ray or computed tomography scan. After the x-ray, the patient is sent for a biopsy. A fragment of the lymph node is taken and examined. A biopsy may reveal Reed Sternberg cells.

Treatment

Sclerosis often affects the female body. The lymph nodes of the mediastinum are damaged, resulting in fibrous cords. Lymphatic tissue becomes divided into nodes. Medical researchers have not yet found an answer to the question of the occurrence of such Hodgkin's disease. It is not known why some people get sick and others do not. But nevertheless, such a disease cannot be called an accident.

There are factors due to which such Hodgkin's disease may develop. Therapy is carried out according to the stage of the disease. If a large number of lymph nodes are affected and the disease spreads to other organs, the patient is prescribed chemotherapy treatment.

After initial chemotherapy-induced remission, the patient may be given an autologous bone marrow transplant.

If the doctor decides to treat with chemotherapy, the stem cells are initially collected and frozen. After aggressive chemotherapy, the cells are reintroduced into the body using an injection method.

The main methods that help cope with such types of Hodgkin's diseases as lymphoma are treatment using combination chemotherapy and radiation therapy. If the forms of the disease have become aggressive, then treatment becomes lengthy.

Patients who suffer from Hodgkin's disease for the first time are prescribed systemic chemotherapy. Medicines act on all organs, because the presence of lymphocytes and lymph tissue is everywhere.

Cytostatics work well in the treatment of drugs together with Rutuximab. Cytostatics are a group of drugs used to induce necrosis in malignant tumors. The drug prevents cells from dividing.

The drug works great thanks to monoclonal antibodies. In general, it affects the tumor with minimal side effects.

Chemotherapy helps achieve good results. As a result, malignant areas of the affected organs are destroyed.

An auxiliary principle for the treatment of Hodgkin's disease is the methods of radiation therapy, although these methods have proven themselves to be positive. The disease can be cured with radiation therapy if it is in the first stage.

In some cases, surgical treatment is suggested. Tumor tumors are excised from patients. Typically, surgical interventions are rarely performed; radiation is performed for direct medical indications.

Biological therapy is used, which includes:

  • immune therapy;
  • gene therapy;
  • angiogenesis inhibitors, which prevent the growth of new tumor formations in Hodgkin's disease.

Lymphoma prognosis

Viruses called Epstein Barr cause half of Hodgkin's diseases. The prognosis for this disease is favorable.

Due to the fact that sclerosis of this type is well treated, 80% of patients have a five-year survival rate. In children this figure is 90%. If immunotherapy is combined with standard therapy, the survival rate will rise even further.

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Interesting about the five-year survival rate... If the girl is 20, then in five years everything will be done. A five-year forecast is a favorable forecast.

The question is, of course, interesting... I am 57 years old. I have, at best, 5 years left.

I got sick at the age of 18. Now I am 42 years old.

I think there's just a typo here. I read that after 5 years of remission, a person is considered completely healthy!

Remission came after 8 years and all the doctors call me unique, so all this does not correspond to the article

The materials on the site are for informational purposes only, consultation with a doctor is required!

Classic Hodgkin's lymphoma nodular sclerosis

Hodgkin lymphoma is a tumor disease that is thought to develop from abnormal lymph cells. The disease was named after Thomas Hodgkin. The English physician Hodgkin discovered this disease in 1832, long before anything became known about the existence of lymphocytes and their function. All other types of lymphoma are called non-Hodgkin lymphomas. They originate from either altered B lymphocytes (more common) or T lymphocytes (less common).

What kind of disease is this

Hodgkin's lymphoma (also called Hodgkin's disease, or lymphogranulomatosis, cancer of the lymph nodes) is a malignant disease of the lymphatic system [lymphatic system]. Lymphomas are different. This disease is malignant.

The name “malignant lymphoma” literally means “malignant enlargement of the lymph nodes.” This medical term unites a large group of cancer diseases that begin in the cells of the lymphatic system (lymphocytes). The main symptom is enlarged lymph nodes (lymphoma).

Malignant lymphomas are divided into two main types: Hodgkin lymphoma (abbreviated as HL) and non-Hodgkin lymphomas (abbreviated as NHL). Hodgkin's lymphoma is named after the physician and pathologist Thomas Hodgkin, who first described the disease. The type of lymphoma can only be determined after examining a sample of the affected tissue.

Hodgkin's disease occurs due to a malignant change (mutation) of B lymphocytes. These are a group of white blood cells (blood cells) that are found primarily in lymphatic tissue. Therefore, Hodgkin lymphoma can appear anywhere there is lymphatic tissue. Lymphomas most often occur in the lymph nodes (lymph nodes), but they can also affect other organs such as the liver, bone marrow, lungs or spleen. As a rule, this happens in the later stages of the disease. If Hodgkin's lymphoma is not treated, it is fatal in most cases.

Symptoms

Most patients consult a doctor with painless enlarged cervical lymph nodes. However, pain in the affected areas can occur after drinking alcohol, which is one of the early signs of the disease, although the mechanism of pain is unclear. Another manifestation of the disease develops when the tumor spreads through the reticuloendothelial system into adjacent tissues. It is characterized by the early appearance of intense itching. Common symptoms are fever, night sweats, spontaneous weight loss (>10% of body weight in 6 months), and there may be signs of damage to internal lymph nodes (mediastinal or retroperitoneal), visceral organs (liver) or bone marrow. Splenomegaly is often present, and hepatomegaly may develop. Sometimes there is Pel-Ebstein fever (alternating between elevated and normal body temperatures; the appearance of high body temperature for several days, then in the next few days or weeks a change to normal or decreased temperature). As the disease progresses, cachexia occurs.

Bone involvement is often asymptomatic, but vertebral osteoblastic lesions (elephant vertebrae) and, less commonly, pain due to osteolytic lesions and compression fractures may occur. Intracranial lesions, as well as gastric and skin lesions, are rare and suggest the presence of HIV-associated Hodgkin lymphoma.

Local compression by tumor masses often causes symptoms such as jaundice caused by intrahepatic or extrahepatic bile duct obstruction; swelling of the legs due to obstruction of the lymphatic ducts in the groin or pelvis; shortness of breath and hoarse breathing, with tracheobronchial compression; pulmonary abscesses or cavities due to infiltration of the pulmonary parenchyma, which may simulate lobar consolidation or bronchopneumonia. Epidural invasion can compress the spinal cord and cause paraplegia. Horner's syndrome and laryngeal paralysis can be caused by compression of the sympathetic cervical and recurrent laryngeal nerves by enlarged lymph nodes. Neuralgia can be a consequence of compression of the nerve root.

How long do they live?

How long do people with Hodgkin's lymphoma live? Today, with timely and high-quality treatment (inpatient and outpatient), the prognosis is considered conditionally favorable. In 85% of all cases, it is possible to achieve remission within 5 years. Slightly less often, remission is achieved at 10 years or longer. Relapses occur in approximately 35% of all cases. In general, many people live with this diagnosis to a very old age.

Diagnosis

The diagnosis of lymphoma is made solely by histological examination of lymph nodes obtained through surgery, called a biopsy of the affected lymph nodes or tumor. The diagnosis of lymphoma is considered proven only if specific multinucleated Sternberg cells (synonyms - Berezovsky-Sternberg or Sternberg-Reed cell) are found during histological examination. In complex cases, immunophenotyping is necessary. Cytological examination (puncture of a tumor or lymph node) is usually not enough to establish a diagnosis.

Lymph node biopsy

General blood analysis

Blood chemistry

X-ray of the lungs – required in frontal and lateral projections

Computed tomography of the mediastinum to exclude enlarged lymph nodes in the mediastinum, invisible with conventional radiography, in the first case and lesions of the lung tissue and pericardium

Ultrasound examination of all groups of peripheral, intra-abdominal and retroperitoneal lymph nodes, liver and spleen, thyroid gland with large lymph nodes of the neck

Trephine biopsy of the ilium to exclude bone marrow lesions

Bone scanning and, if indicated, bone radiography

Nodular sclerosis

Nodular sclerosis is the most common form of Hodgkin lymphoma, predominant in women and usually affecting the lower cervical, supraclavicular and mediastinal lymph nodes. The disease occurs in young people and is characterized by a good prognosis (especially in stages I or II), as well as three morphological features that distinguish it from other forms of Hodgkin lymphoma:

In most cases, in the affected lymph node, the lymphoid tissue in the form of isolated nodes is divided by either more or less developed ring-shaped fibrous layers;

In addition to lymphocytes, this tissue may contain eosinophils and histiocytes in varying quantities; “classical” Reed-Sternberg cells are rare;

In the lymphoma tissue, one of the variants of tumor differentiation of Reed-Sternberg cells is found - lacunar cells (syn. Hodgkin cells), also having large sizes, one lobulated nucleus with small nucleoli and developed pale cytoplasm; the latter often undergoes wrinkling, due to which an optically empty space is created around the cell - a lacuna; The immunophenotype of lacunar cells is the same as that of Reed-Sternberg cells: they express CD 15, CD30, but usually do not contain B- and T-cell antigens.

Causes

The exact cause of lymph node cancer has not been identified. However, scientists identify risk factors that contribute to the onset of the disease:

Carriage of the Epstein-Barr virus;

Genetic abnormalities, hereditary predisposition;

Radiation exposure: work in nuclear power plants, excessive exposure to x-rays;

Decreased immunity as a result of radiation therapy, chemotherapy, organ transplantation, HIV;

Inhalation of carcinogens when working with hazardous chemicals;

Taking corticosteroid drugs.

Chemotherapy

Each type of drug has its own effect on cells affected by lymphoma. Combining different types of medications can increase the effectiveness of treatment. As a rule, such combinations are designated by an abbreviation made up of the first letters of the names of the products. The most effective combinations for the treatment of HL:

ABVD. Doxorubicin, bleomycin, vinblastine, dacarbazine;

BEACOPP. Blenoxan, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine, prednisolone;

Stanford V. Meclorethamine, Adriamycin, Cytoxan, Oncovin, Blenoxan, Etoposide, Prednisolone.

The ABVD combination is used both for the treatment of stage I and II HL (in combination with radiation therapy), and for stages III and IV of the disease (during a longer period of treatment). ABVD is the main combination for the treatment of most adult patients, due to its effectiveness and the fact that the course has a lower risk of serious side effects (infertility and leukemia) than other combinations. Among patients whose disease is at stages I and II, the number of those cured is more than 95%.

Patients with stage I and II HL typically undergo only two ABVD cycles over several weeks, followed by reduced-dose radiation therapy. If they are at high risk, four to six cycles may be required before radiation therapy. The general course of treatment lasts from six to eight months.

Patients susceptible to stage III and IV HL undergo 6-8 courses of chemotherapy, such as ABVD or BEACOPP. BEACOPP has a good cure rate but is associated with an increased risk of leukemia and secondary cancers. BEACOPP can be used to treat aggressive subtypes of HL.

Stanford V and other drug combinations can be used with or without radiation therapy. One cycle takes less time than ABVD, but the periods between cycles are also reduced.

Consequences and side effects

Some chemotherapy side effects (such as fatigue and depression) may last for several years after treatment. Other diseases (such as heart disease and other types of cancer) may not manifest themselves until several years after the end of the treatment course.

Long-term and late effects of chemotherapy include:

Thyroid dysfunction;

Post-traumatic stress disorder;

Loss of ability to concentrate;

Classical

Histologically, classical Hodgkin lymphoma is composed of varying numbers of Reed-Strenberg cells, mononuclear Hodgkin cells and their variants (H/RS), located among small lymphocytes (mainly T cells), eosinophils, neutrophilons, histiocytes, plasmacytes, background fibroblasts and collagen. The four subtypes of classical Hodgkin lymphoma, defined largely from the background population, have significant differences in clinical presentation and features such as association with Epstein-Barr virus. The immunohistochemical and molecular hevetic characteristics of H/RS cells are identical in the subtypes of Hodgkin lymphoma.

Classic Hodgkin lymphoma can occur at any age, including early childhood. It is characterized by a bimodal age distribution with an early peak of incidence between 15 and 35 years and a later, less clearly defined, peak in old age. An increased incidence of classical Hodgkin lymphoma is observed in patients with a history of infectious mononucleosis and HIV infection.

In classical Hodgkin lymphoma, the central groups of lymph nodes (cervical, mediastinal, axillary, inguinal and para-aortic) are typically affected; mesenteric nodes are less common. In 60 g of patients with nodular sclerosing Hodgkin lymphoma (NSHL), mediastinal tumors occur with involvement of the lymph nodes and thymus. Tumor involvement of the spleen is found in 20% of patients and has been shown to cause further hematogenous spread to the liver and bone marrow. Primary lesions of Waldeyer's ring (palatine and pharyngeal tonsils) and the gastrointestinal tract are rare.

Stage 1: involvement of one group of lymph nodes or lymphoid structure.

Stage 2: involvement of two or more groups of lymph nodes or lymphoid structures on one side of the diaphragm.

Stage 3: damage to groups of lymph nodes or lymphoid structures on both sides of the diaphragm.

Stage 4: involvement of extra-nodal areas, such as the liver and bone marrow.

In children

The main symptom of Hodgkin lymphoma in a child is enlarged lymph nodes (lymphadenopathy).

Among extranodular localizations, it should be noted (up to a quarter of cases) damage to the spleen; the pleura and lungs are often involved in the process. Damage to any organs is possible - bones, skin, liver, bone marrow. It is extremely rare to observe tumor growth into the spinal cord and specific infiltration of the kidneys and thyroid gland.

Numerous treatment programs for Hodgkin lymphoma have been developed in different countries. Their fundamental elements are radiation therapy and polychemotherapy using a relatively narrow range of drugs. It is possible to use radiotherapy alone, chemotherapy alone, or a combination of both methods. Radiotherapy and chemotherapy programs for lymphogranulomatosis are constantly being improved: their effectiveness increases, immediate and late toxicity decreases without the development of resistance. Treatment tactics for Hodgkin lymphoma are determined by the stage of the disease and the age of the patient.

Hodgkin's disease in children has a different prognosis; it primarily depends on the stage at which treatment was started. With local forms of lymphogranulomatosis (IA, IIA), complete recovery is possible in 70-80% of children, although complete remission is achieved in 90%. We can talk about recovery only 10 years after the completion of a successful course of primary treatment. Most relapses occur in the first 3-4 years after the end of therapy.

Mixed cell variant

The mixed-cell variant of Hodgkin's lymphoma, according to various authors, accounts for 15 to 25% of cases of all classical variants of Hodgkin's lymphoma.

Involvement of the mediastinal lymph nodes is observed in 40% of cases. The histological picture is characterized by diffuse growth of the tumor, represented mainly by classical Berezovsky-Sternberg cells among a reactive background, often localized in the paracortical zone between follicles with light atrophic or regressively transformed germinal centers (the so-called interfollicular subvariant of the mixed-cell variant of Hodgkin lymphoma).

The capsule of the lymph node is not thickened; areas of diffuse fibrosis can be detected without the formation of wide collagen strands. Necrosis is uncommon. The cellular composition of the reactive background is represented by a “mixed cell” population with the presence of plasma cells, eosinophils, macrophages, epithelioid histiocytes, neutrophilic leukocytes, and lymphocytes mainly with a T-cell phenotype (CD3+, CD45R0+).

One of the listed components may predominate. T lymphocytes form rosette-like structures around large tumor cells. Epithelioid histiocytes can form granuloma-like clusters, which are described as a subvariant rich in epithelioid cells. Among tumor cells, “mummified” cells can be observed in significant numbers.

Hodgkin's lymphoma

Lymphoma what kind of disease is it? This is damage to the lymphatic system of the body by various malignant tumors, that is, the lymph nodes along with the small vessels that unite them. With lymphoma, tumor cells begin to divide indefinitely, and their descendants begin to populate the lymph nodes (LNs) and/or internal organs and disrupt their normal functioning.

More than 30 diseases with different clinical manifestations, course and prognosis are united by the concept “lymphoma”. The main types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma. They belong to lymphoproliferative diseases that affect any organ, have different histological and clinical symptoms and prognosis. B- and T-cell lymphoproliferative diseases (with the exception of B- and T-cell leukemias) are combined into a group - “non-Hodgkin lymphomas”.

The primary development of a lymphoproliferative disease in the bone marrow is called leukemia (eg, CLL). The primary occurrence of a tumor in lymphoid tissue outside the bone marrow is called lymphoma. If lymphoma arises from the lymphoid tissue of an internal organ: brain, liver, colon and others, then the name of the affected organ is added to the word “lymphoma,” for example, “stomach lymphoma.”

Hodgkin's lymphoma, what is it?

The disease was first described by Thomas Hodgkin in 1832, and the name was introduced into the WHO in 2001. Hodgkin's lymphoma has a second name - lymphogranulomatosis. It affects the bodies of men more often than women.

Hodgkin's lymphoma, what is this disease? Before the classification of lymphomas was created, it was believed that Hodgkin lymphoma has two peaks of development - at age and after 50 years. When reviewing histological preparations, immunophenotyping began to be used. It indicated the absence of a second peak or its slight development. Therefore, most of the histological means after retrospective analysis were classified as the second type of lymphoma - non-Hodgkin large cell.

Hodgkin's lymphoma, previously incurable, after timely detection and treatment with modern methods and drugs, is cured in 85% of cases or stable remission can occur.

What is Hodgkin's lymphoma? Specific signs of the disease appear after an early stage. After damage to the lymph nodes, lymphogranulomatosis or Hodgkin's lymphoma tends to spread and invade any organ, accompanied by severe symptoms of intoxication. The clinical picture of Hodgkin lymphoma is determined by the predominant damage to an organ or system.

Informative video

Causes of Hodgkin's lymphoma development

Hodgkin's lymphoma has not yet been fully studied, but scientists have identified a number of provoking factors that provoke the disease. The causes of Hodgkin's lymphoma are as follows:

  • weakening of the immune system due to HIV infection, organ transplantation, forced use of medications and the presence of rare diseases that negatively affect the immune system;
  • infection with the Epstein-Barr virus, which causes glandular fever.

According to research, Hodgkin lymphoma, the causes and symptoms of the disease are not an infectious disease and are not transmitted from a patient to another person. The exception is twins, since they have a higher risk of the disease than other family members if there is a genetic factor.

Signs and symptoms of Hodgkin's lymphoma

Hodgkin's lymphoma: signs of the disease:

  • LNs can be felt on the surface of the skin in 90% of patients (including children): they swell but do not hurt. They accumulate in the areas: neck, back of the head, under the armpits, above the collarbones or groin, perhaps their manifestation in several areas at once;
  • in the presence of lesions in the lymph nodes of the chest, lungs or pleura - manifested by shortness of breath and chronic cough;
  • when the lymph nodes of the peritoneum or other organs are affected: the spleen or liver - manifested by back pain, a feeling of heaviness in the abdomen or diarrhea;
  • when lymphoma cells enter the bone marrow, symptoms are characterized by pale skin (anemia) due to a lack of red blood cells;
  • with bone damage - pain in the bones and joints.

Symptoms of Hodgkin lymphoma appear slowly and increase over a month or six months. Each patient has his own symptoms with different manifestations. In children, Hodgkin's lymphoma can hide behind a common infection. Or, conversely, the growth of lymph nodes during a viral infection is mistaken for lymphoma. It is necessary to consult a doctor in any case for examination, especially if you have a prolonged cough.

When the cervical supraclavicular lymph nodes (usually on the right) increase by 60-75%, Hodgkin's Lymphoma is suspected, the symptoms of which do not yet disturb the patient's well-being. The lymph nodes will be mobile, densely elastic, not fused to the skin, and sometimes painful.

In the case of gradual growth of lymph nodes and merging into large conglomerates, Hodgkin lymphoma, symptoms manifest themselves as pain, especially after drinking alcohol.

Enlarged mediastinal lymph nodes in 15-20% of patients indicate the onset of the disease. This can only be discovered by chance during fluorography for another reason, when many conglomerates can be seen in the image. At this time, cough, shortness of breath and compression of the superior vena cava may appear, which is noticeable by swelling and cyanosis of the face, less often by pain in the chest.

Isolated cases of pain in the lumbar region due to isolated lesions of the paraortic lymph nodes (retroperitoneal, located along the spine) indicate the onset of pathology.

Symptoms of Hodgkin's lymphoma also include acute fever, increased night sweats, and rapid weight loss, which occurs in 5-10% of patients in the first stage of the disease. Their lymph nodes increase later, and early leukopenia and anemia (anemia) accompany the disease. Losing weight for no reason is a clear symptom of the disease.

Lymphoma is characterized by a variety of fevers. With daily short-term rises in temperature, chills appear, then the patient sweats excessively and it all ends.

Sometimes Indomethacin or Butadione is taken for fever. Sweating occurs in all patients. In severe cases of the disease

With advanced manifestations of lymphoma, all lymphoid organs, systems and other organs in the body are affected. In 25-30%, the spleen is affected in the presence of clinical stages 1 and 2, which are diagnosed before splenectomy. Waldeiro's ring: tonsils and lymphoid tissue of the pharynx are rarely affected by lymphoma.

If lung tissue is damaged:

  • cells from the mediastinum grow infiltratively;
  • individual foci or diffuse infiltrates develop that can disintegrate and form cavities;
  • fluid accumulates in the pleural cavities.

Specific damage to the pleura can be seen on x-ray. Cancer cells are found in the pleural fluid: lymphoid, reticular, and Berezovsky-Sternberg. The pleura is affected by lymphogranulomatosis with the presence of enlarged mediastinal lymph nodes or lesions in the lung tissue. From the mediastinal lymph nodes, the tumor grows into the pericardium, myocardium, trachea and esophagus.

The disease affects the skeletal system in 20% of patients, affecting the vertebrae, sternum, hip bones, ribs, and tubular bones (rarely). When the skeletal system is involved in the process, pain appears, which is a clear sign of lymphogranulomatosis.

When the bone marrow is damaged, the level of platelets and leukocytes in the blood decreases, anemia begins, or symptoms do not appear at all.

The compensatory capabilities of the liver prevent cancer from being detected in the early stages. With Hodgkin's lymphoma, organ enlargement occurs, alkaline phosphatase activity increases, and serum albumin decreases.

When a tumor grows from the affected lymph nodes or the gastrointestinal tract is compressed, it suffers secondarily. When the stomach and small intestine are affected, the process spreads through the submucosal layer without the formation of ulcers.

The central nervous system is affected: in the spinal cord, cancer cells accumulate in the membranes of the brain, which leads to serious neurological disorders or complete paralysis.

A small rash with Hodgkin lymphoma causes itching in 25-30% of patients. Itching can be from dermatitis or in areas of enlarged lymph nodes throughout the body. Patients suffer painful itching, they lose sleep and appetite, and mental disorders occur.

There are no specific changes in blood tests for lymphoma. Most often, patients have a moderate level of leukocytes. Only the later stages of lymphoma are characterized by a decrease in lymphocytes in the blood.

Informative video: lymphogranulomatosis

Types of Hodgkin's lymphomas

Based on the appearance of tumor cells under a microscope, five forms of Hodgkin's disease are identified. Four types are united by “classical Hodgkin lymphoma.”

In accordance with the WHO classification (2008), the following are considered morphological variants of lymphogranulomatosis:

  1. Hodgkin's lymphoma, nodular type with lymphoid predominance.
  2. Classic Hodgkin's lymphoma. It includes:
  • classic Hodgkin lymphoma – lymphoid predominance;
  • classic Hodgkin's lymphoma - nodular sclerosis (nodular form);
  • classical Hodgkin's lymphoma - mixed cell;
  • classic Hodgkin's lymphoma - lymphoid depletion.

Nodular type of lymphoid predominance

This type accounts for 5% of all types of Hodgkin lymphoma and is more common in men, starting from childhood and later in life. Due to the long course of lymphadenopathy, pathology is detected at stages I or II in 80% of patients. Some patients are cured with excisional therapy.

It differs from classical lymphoma in behavior, histological structure, and characteristics: molecular genetic and immunohistochemical. VU of the nodular type with lymphoid predominance, few plastic L&H cells, many small B cells, nodular signs.

The cervical, axillary and inguinal lymph nodes (less commonly) are affected. Lymphoma is found even less often in other groups of lymph nodes.

In most patients, the course of the disease can be sluggish, relapses often occur, but progression and death are rare - in 5% of patients.

In such cases, it is necessary to reconsider and reassess the nature of relapses, since the diagnosis may be misdiagnosed. The lymphoid-predominant variant of HL and the T-cell/histiocyte-rich B-cell lymphoma are often confused. To find out, you need to study the nodules in the biopsy.

In the nodular type with lymphoid predominance, there is dense fibrous tissue around the nodules of Hodgkin's tissue.

Classic Hodgkin's lymphoma

Histologically, classical lymphoma is arranged in different numbers of cells:

  • Reed-Strenberg;
  • mononuclear Hodgkin's;
  • their variants (H/RS), located among small lymphocytes (mainly T cells);
  • eosinophils, neutrophilons, histiocytes, plasmacytes, background fibroblasts and collagen.

The four subtypes differ in clinical presentation and association with Epstein-Barr virus. According to immunohistochemical and molecular genetic characteristics, H/RS cells are identical. In lymphoid predominance there are few Reed-Sternberg cells, many B cells, and reticular sclerosis predominates.

Classic Hodgkin's lymphoma affects people from early childhood, and the disease peaks in old age. People with infectious mononucleosis and HIV infection often get sick. In classical Hodgkin lymphoma, lesions are found in the central groups of lymph nodes:

Sclerosing nodular Hodgkin lymphoma

Nodular sclerosis of Hodgkin's lymphoma is found in 60%-67% of patients, most often mediastinal tumors affecting the lymph nodes and thymus. The spleen is affected by the tumor in 20% of patients, then spreads hematogenously to the liver and bone marrow. Less commonly, Waldeyer's ring is affected (in the area of ​​the palatine and pharyngeal tonsils). The development of nodular sclerosis occurs gradually. Namely, they are affected by:

  • at the first stage - one group of lymph nodes or lymphoid structure;
  • at the second stage - two groups (or more) of lymph nodes or lymphoid structures on one side of the diaphragm;
  • at the third stage - lymph nodes or lymphoid structures on both sides of the diaphragm;
  • at the fourth stage - extra-nodal areas, such as bone marrow or liver.

Classic Hodgkin lymphoma is defined by systemic manifestations: fever, profuse night sweats and weight loss and is classified as “B-symptoms”, which gives a poor prognosis.

Mixed cell lymphoma

The clinical picture of follicular lymphoma is determined by the cytological characteristics of the cells that form the follicles. With a sluggish course, lymphadenopathy is detected several years before diagnosis. The patient may feel well for 5 years or longer after diagnosis. But there may be a transient manifestation of lymphoma, which requires urgent treatment. It is now known that follicular lymphoma with a low degree of malignancy is capable of transformation. It develops into highly malignant diffuse large cell lymphoma.

Follicular mixed-small cell lymphoma consists of small cells with a split nucleus and large cells. A mixed infiltrate is noticeable in Reed-Sternberg cells. After its treatment, a long-term remission occurs without relapses. The prognosis for cure is very high.

Lymphoid depletion

The rarest type of classic lymphoma is lymphoid wasting. Occurs in 5% of people over 50 years of age,

The tumor is dominated by Reed-Sternberg cells. Small inclusions of lymphocytes are noticeable between them. In diffuse sclerosis, the growth of coarse strands of connective tissue fibers sharply predominates, in which amorphous protein masses fall out. The level of lymphocytes in tumor cells is constantly decreasing.

Stages of Hodgkin's lymphomas

When establishing the stages of Hodgkin's lymphoma, anamnesis, clinical examination, biopsy data, as well as examination results obtained visually were used; the manifestation of the disease was distinguished: lymphatic (nodal) and extralymphatic (extranodal).

Lymphatic structures consist of:

  • lymph nodes;
  • Waldeyer rings;
  • spleen;
  • appendix;
  • thymus gland;
  • Peyer's patches.

The symbol E denotes an extralymphatic (extranodal) manifestation, in which nonlymphatic structures and tissues are affected.

Classification by stages

  1. Hodgkin's lymphoma stage 1. One lymphatic zone or structure is affected (I). One lymphatic zone or structure is affected, and adjacent tissues are involved (IE). One extralymphatic organ (IE) is locally affected.
  2. Hodgkin's lymphoma stage 2. Localized, one extralymphatic organ and its regional lymph nodes are affected with damage (or without involvement) of other lymphatic zones on one side of the diaphragm (IIE). Hodgkin's lymphoma stage 2: the prognosis for survival for 5 years is 90-95%. Hodgkin's lymphoma stage 3. The lymph nodes on both sides of the diaphragm are affected (III). The pathological process is combined with localized damage to one extralymphatic organ or tissue (IIIE), with the spleen (IIIS), or with damage to both (IIIE + S). Hodgkin's lymphoma stage 3: survival prognosis – 65-70% (according to various sources).
  3. Hodgkin's lymphoma stage 4. One or more extralymphatic organs are subject to disseminated (multifocal) damage with or without damage to the lymph nodes; The extralymphatic organ is isolated and involves a distant (non-regional) lymph node. Hodgkin's lymphoma stage 4: prognosis for survival for 5 years – 55-60%.

Stages of Hodgkin's lymphomas

Important! If the liver, bone marrow, or lung are affected (except for direct spread of the tumor from the lymph nodes), stage IV is diagnosed with diffuse extranodal spread of the disease.

If remission exceeds the five-year barrier, then the lymphoma is considered cured. However, studies of the body should be carried out annually, since relapse can occur and remission after a year. A long-term consequence of HL after chemotherapy – cardiomyopathy (myocardial damage) – is possible.

Diagnosis of the disease

Diagnosis of Hodgkin lymphoma is carried out as follows:

Diagnosis of submandibular lymph nodes

  • the anamnesis is examined for the presence of symptoms of intoxication;
  • establish “alcohol” pain zones in the affected areas;
  • the growth rate of lymph nodes is determined;
  • carefully palpate: all peripheral groups of lymph nodes (submandibular and cervico-supraclavicular, subclavian and axillary, iliac and inguinal, popliteal and femoral, elbow and occipital), liver and spleen;
  • An ENT doctor examines the nasopharynx and palatine tonsils;
  • An excisional biopsy is performed from the earliest lymph node that appears. It is removed completely provided there is no mechanical damage.

Histological examination confirms the diagnosis, since only with a specific description of the diagnostic Berezovsky-Reed-Sternberg cells and accompanying cells is the diagnosis considered final. Based on the clinical picture, radiograph, presumptive histological or cytological conclusion without a description of the cells, the diagnosis will be considered controversial.

For histological examination, inguinal lymph nodes are not taken if other groups of them are involved in the process. The initial diagnosis is not determined by needle biopsy alone. Therefore, the list of diagnostic measures continues:

  • Ultrasound of peripheral lymph nodes: cervical, supraclavicular and subclavian, axillary, inguinal and femoral, peritoneum and pelvis. In this case, the liver, spleen, para-aortic and iliac lymph nodes are examined;
  • CT scan of the neck, chest, abdomen and pelvis;
  • according to indications – osteoscintigraphy;
  • radiography of bones (if patients complain of pain and changes detected on scintigrams);
  • study of blood group and Rh factor, general blood test, including the level of red blood cells, platelets, hemoglobin, leukocyte formula, ESR;
  • blood test using a biochemical method for the content of creatinine, bilirubin, urea, total protein, AST, ALT, LDH, alkaline phosphatase;
  • study of the amount of thyroid hormones if the cervical lymph nodes are affected and irradiation of the neck is planned;
  • bone marrow biopsy, in which trepanobiopsy of the iliac wing is performed, since cytological examination does not definitively confirm the diagnosis;
  • gallium scintigraphy.

If Hodgkin's lymphoma is finally determined, the diagnosis is formulated indicating the stage, B-symptoms (if any), areas of massive damage, involvement of extranodal zones and the spleen. The most significant risk factors that determine the prognosis of the disease are:

  • A – massive mediastinal lesion with mediastinal-thoracic index (MTI) ≥ 0.33. MTI is determined by the ratio of the maximum width of the mediastinum and the chest (level of 5-6 vertebrae of the thoracic zone);
  • B – extranodal lesion;
  • C – ESR ≥ 50 mm/h at stage A; ESR≥30 mm/h at stage B;
  • D – damage to three or more lymphatic zones.

Treatment of Hodgkin's lymphoma

Treatment of Hodgkin's lymphoma is carried out taking into account the stages and risk factors of the disease. Modern therapy is correlated with accurate verification of the tumor subvariant. If the diagnosis is simplified and a “group” diagnosis is formed (for example, “high-grade lymphoma”), then the opportunity to provide therapeutic care to the patient worsens.

Chemotherapy for Hodgkin lymphoma and radiation are included in programs depending on the nosological diagnosis and stage of the disease. The following become cytostatic agents under the CHOP program:

Chemotherapy for Hodgkin lymphoma is carried out intensively in the absence of leukemia. In this case, a subsequent transplantation of autologous bone marrow is carried out, which is prepared in advance for the patient before intensive treatment.

Complete remission after the use of modern programs occurs in 70-90% of patients with primary lymphoma. Relapse-free survival at 20 years with complete remission after the first stage of treatment exceeds 60%.

As an independent treatment, the surgical method is not used for the diagnosis of Hodgkin lymphoma. Radiation therapy, as an independent regimen, is used in a single dose of 1.5-2.0 Gy. The areas of clinically affected lymph nodes are irradiated with a total focal dose of Gy if only radiation therapy is used. Preventive irradiation zones receive –Gr. In Russia they use the wide-field irradiation technique.

When diagnosed with Hodgkin lymphoma, primary patients are not treated with monochemotherapy. The exception is weakened elderly patients with bone marrow hypoplasia after multiple courses of chemotherapy. The effect of monochemotherapy is 15-30%, but it also ensures satisfactory health for some time and inhibits the progression of Hodgkin lymphoma in patients who are not able to undergo modern methods of therapy.

Chemotherapy drugs, regimens and courses used for the treatment of Hodgkin lymphoma

  • Most often, treatment is carried out in mono mode with Vinblastine. Dose – 6 mg/kg once a week. The interval is extended to 2-3 weeks after 3-4 injections.
  1. 100 mg Natulan, total dose – 6-8 g;
  2. 10 mg Chlorambucil (5 days), total dose – mg.
  • Primary patients with Hodgkin's lymphoma are treated with combined chemoradiotherapy according to the ABVD regimen. The drugs included in the regimen are administered intravenously for 1-14 days. Namely, they introduce (with an interval of 2 weeks between courses):
  1. 375 mg/m² - Dacarbazine;
  2. 10 mg/m² - Bleomycin;
  3. 25 mg/m² - Doxorubicin;
  4. 6 mg - Vinblastine.
  • From the 15th day after the last administration of drugs, the next cycle of treatment begins. They prefer treatment according to the BEACOPP regimen in a dose escalation regimen:
  1. day one - 650 mg/m² Cyclophosphamide and 25 mg/m² Doxorubicin are administered intravenously;
  2. day 1-3 (three) days – 100 mg/m² Etoposide is administered;
  3. day eight - 10 mg/m² Bleomycin and 1.4 mg/m² Vincristine are administered;
  4. taken orally for 1-7 days. – 100 mg/m² Procarbazine and 2 weeks 40 mg/m² Prednisolone.

The break before the next course is 7 days (after taking Prednisolone) and 21 days after the start of the course. Radiation therapy is carried out after chemotherapy.

High-dose chemotherapy for Hodgkin lymphoma (except for patients with stage IA with no risk factors and a histological variant of lymphoid predominance, who receive only one radiation therapy to the affected area - SOD 30 Gy) is used, for example, according to the following schemes:

  • Doxorubicin – 25 mg/m² by intravenous infusion (start-up), Bleomycin – 10 mg/m2 (in 10 minutes) and Vinblastine – 6 mg/m2 (start-up) - on days 1 and 15;
  • Dacarbazine – 375 mg/m² intravenously on days 1-15.

The break is 2 weeks, the next course begins on the 29th day from the start of the previous course.

  • Cyclophosphamide – 650 mg/m² orally (zamin) on the first day;
  • Doxorubicin – 25 mg/m² orally (zamin) on the first day;
  • Etoposide – 100 mg/m² intravenously (zamin) on days 1-3;
  • Procarbazine – 100 mg/m² orally on days 1-7;
  • Prednisolone – 40 mg/m² orally on days 1-14;
  • Vincristine – 1.4 mg/m² – injected intravenously on the 8th day (maximum dose – 2 mg);
  • Bleomycin - 10 mg/m² orally on the 8th day.

The next course begins 7 days after using Prednisolone or on the 22nd day from the start of the first course.

  • Cyclophosphamide – 1250 mg/m² orally (in 60 minutes) – on the 1st day;
  • Doxorubicin – 35 mg/m² IV (zamin) on the 1st day;
  • Etoposide – 200 mg/m² IV (in 60 minutes) on days 1-3;
  • Procarbazine - 100 mg/m² on days 1-7;
  • Prednisolone – 40 mg/m² orally on days 1-14;
  • Vincristine – 1.4 mg/m² intravenously on the 8th day (no more than 2 mg);
  • Bleomycin – 10 mg/m² IV (zamin) on the 8th day;
  • Granulocyte colony-stimulating factor (G-CSF) subcutaneously on days 8-14.

Start the next course 7 days after completing Prednisolone or on the 22nd day from the start of the previous course.

  • BCNU (Carmustine) - 300 mg/m² on day 1;
  • Etoposide - mg/m² on days 2-5;
  • Cytosar - 200 mg/m² every 12 hours on days 2-5;
  • Melphalan - 140 mg/m² on the 6th day;
  • Automyelotransplantation and/or return of peripheral blood hematopoietic progenitor cells on day 8.
  • Cyclophosphamide 1.5 g/m² on days 1-4;
  • Etoposidom mg/m² every 12 hours, 6 injections on days 1-3;
  • BCNU (Carmustine) 300 mg/m² on day 1;
  • Automatic heliotransplantation and/or return of peripheral blood hematopoietic progenitor cells on the 5th day.

The next mandatory component of treatment is radiation therapy. Only areas with the original tumor process are irradiated. If standard chemotherapy regimens are ineffective, high-dose chemotherapy is performed and hematopoietic stem cells are transplanted.

For the treatment of patients with limited stages, a treatment program is used: 2-3 courses of ABVD and irradiation of areas with a tumor process - a dose of 30 Gy. The next course is in 2 weeks.

Informative video: modern approaches in the treatment of relapsed and resistant forms of Hodgkin lymphoma

Alternative therapy for Hodgkin's lymphoma

Treatment of Hodgkin's lymphoma with folk remedies is included in general therapy.

Treatment with celandine

Celandine for lymphoma

Celandine is harvested in April-May during flowering; it is advisable to collect the plant away from roads and polluted areas. The roots need to be cleaned with a knife; there is no need to wash or crush the roots. Only last year's dry and dirty leaves are thrown away. Cut the plant into pieces of 2-3 mm. on a clean board where meat was not cut.

Fill a sterilized 3-liter bottle with chopped celandine and compact it with a clean rolling pin. Bandage the neck of the bottle with gauze and leave to infuse for 3 days at room temperature in a dark place. Using a clean wooden stick, pierce the contents of the jar to the bottom 3-5 times a day, like cabbage when fermenting.

On the 4th day, use a juicer to extract the juice. It will turn out to be about ml. celandine juice. Place the juice in sterilized bottles (jars) in a dark place for 2 days. Place the plant cake back into the bottle and add vodka or strong moonshine - ml. Leave for 8-9 days in the dark. Then separate the tincture from the grounds and let it infuse like juice. After 2 days, the juice will become the color of tea, or cloudy tea. Pour juice and infusion into bottles. Close them with plastic stoppers and stick a label with the date of manufacture.

Celandine juice will be needed to treat Hodgkin's lymphoma (or any cancer). For treatment take:

  • 1-2 degree – 1 tbsp. l. per 100 ml of milk (fermented milk product) – 1 time at night. You can also drink it with milk – 100 ml. The course will require 0.5 liters or a little more;
  • 3-4 degree – 1 tbsp. l. per 100 ml of milk - 2 times with an interval of 12 hours. You will need 0.7 liters for the course. Consider this technique as the main medicine.

To prevent the disease you will need 350 ml. After each bottle of juice you drink, do a blood test. After the first week, the condition may worsen; after a week there will be an improvement, which will be shown by a blood test.

An additional (concomitant) treatment will be chestnut beer. To prepare it:

  • chestnuts (20-30 pcs.), growing in the park, divide into 2-4 parts and put in a bottle - 3 liters;
  • prepare a mixture of herbs: to celandine (5 tbsp.) add chamomile and calendula, yarrow and coltsfoot - 2 tbsp each. l. Place the collection (1 tbsp) in a gauze bag with a piece of silicon and place it on the days of the bottle;
  • Pour whey over chestnuts and grass, add sour cream (1 tbsp.);
  • tie the neck of the bottle with 2 layers of gauze and place it on the window, but not in direct sunlight;
  • as soon as fermentation begins (after 2-3 weeks), you need to consume 1-2 tbsp./day, adding melt water and honey (sugar) to the bottle.

Viburnum can be a concomitant treatment. A 3 liter bottle is filled with it. 4/5 of the volume and fill with melt water and honey (sugar). For hypertension, viburnum is replaced with dry chokeberry. Drink 1 tbsp each, top up with sweet water. Enough for the autumn-winter season. Once a week it is useful to drink a decoction of wormwood (or wormwood powder - 0.5 tsp), cloves and nut tincture.

Nutrition for malignant Hodgkin lymphoma

Healthy nutrition for lymphoma

Nutrition for Hodgkin lymphoma is aimed at restoring the body and enhancing the immune system after treatment. Products must ensure that the body receives a sufficient amount of calories (proteins) and vitamins. They must participate in hematopoiesis and increase hemoglobin so that the body can fight cancer.

The diet for Hodgkin's lymphoma is developed according to the recommendations of a nutritionist or attending physician. It should be frequent and divided, because after chemotherapy or radiation, patients have no appetite due to nausea and will not eat a large portion. Food should stimulate the appetite by its appearance, emit a pleasant smell, and be at room temperature. Hot and cold dishes will not add to your appetite, nor will tea that is too hot.

Before meals you need to drink a glass of water, juice, compote or warm tea. You can add a small amount of spices, mayonnaise or mustard to second courses to increase your appetite.

At the initial stage of lymphoma, doctors do not prescribe a specific therapeutic diet, limiting only fatty, fried and spicy-salty foods. In the progressive form of the disease, nutrition is structured to maintain the vitality of the body and smooth out the side effects of medications.

There is no single diet for use in cases of severe manifestations of cancer, so that it can be used in practice, since the patient’s metabolism of the body is disrupted: energy, carbohydrate and protein. This disorder is the result of exposure to tumor intoxication, due to which cachexia (exhaustion) occurs.

There is currently no consensus among doctors on the issue of dietary nutrition for lymphomas and other cancers precisely because of the specifics of metabolism. Previously, it was believed that high-calorie foods also fed the tumor, since it was a trap for all nutrients. There are erroneous theories of adhering to partial or complete fasting, eating exclusively juices and excluding water. Other nutritionists suggest minimizing simple carbohydrates coming from honey, sugar, pasta, bread and rolls, semolina and confectionery.

Tumors “feed” on glucose, but with artificial restriction of carbohydrates, gluconeogenesis can be activated, in which glucose is formed from substances that do not contain carbohydrates during the decomposition of fats and proteins. Due to this, hypoglycemic syndrome occurs: the level of glucose in the body becomes below the physiological norm.

A careless diet along with nonspecific reactions of organs to a tumor in lymphoma leads to hypoglycemia. Therefore, correction of metabolic processes using insulin therapy methods is used for this.

Scientists also came to the conclusion that with an excess of vitamin C, the condition of patients worsens during treatment for tumors. Excess vitamins are harmful during chemotherapy and radiation. Leading clinics recommend taking multivitamins with food - 1-3 tablets/day, but no more.

Diet recommendations require a flexible approach and consideration of the patient's condition. For example, during tumor growth, hypernatremia (excess sodium salts) is detected, which retains fluid in the body and causes swelling. At these moments, it is necessary to limit salt intake, not to eat salted and smoked foods, and to increase sources of potassium salts in the diet.

If the patient cannot tolerate food without salt at all, this may affect his appetite. Then snacks that increase appetite are added to the diet: caviar, olives in combination with drugs that remove sodium. If frequent vomiting and diarrhea occur after chemotherapy, then sodium salts should, on the contrary, be increased in the menu.

Modern nutritionists believe that there are no completely prohibited foods for lymphoma, and nutrition for patients should be complete and enjoyable. Their menu should be varied and taste much higher than that of a healthy person.

Consequences of treatment and relapse of the disease

With modern treatment methods, in the last 5-8 years, Hodgkin lymphoma has achieved long-term stable remission (more than 5 years) and a favorable prognosis for more than half of patients from all cases. The smallest group includes patients with stages IA-IIA lymphoma. With a small amount of treatment, life expectancy is 10 years or more. The group includes patients under 40 years of age without risk factors. Complete remission was observed in 93-95% of patients, relapse-free course - in 80-82%, 15-year survival - in 93-98% of patients.

In the presence of risk factors in patients with stage IIA, with stages IIB and IIIA, relapse-free survival for 5-7 years after irradiation is only 43-53%. Patients survive solely due to progressive treatment of relapses.

If Hodgkin's lymphoma is diagnosed, how long do patients in the intermediate prognostic group live? According to studies, the 15-year survival rate was 69%. The risk of recurrence of Hodgkin's lymphoma when receiving radical radiation therapy was 35%, and when receiving combined treatment – ​​16%.

Important fact! If we reduce the volume of radiation therapy and irradiate only the areas of the initial lesion in patients with an intermediate prognosis group, this will not reduce the immediate or long-term results of treatment. The main method of treatment for primary patients with Hodgkin lymphoma with any extent of the disease is combined chemoradiotherapy. Lymphoma prognosis for 5 years is 80-90%.

Patients from the unfavorable prognostic group are treated with polychemotherapy. Complete remission from effective treatment was 60-80%. Recurrence of Hodgkin's lymphoma after the introduction of new drugs (Etoposide) into the first-line program and reduction of intervals between doses was 4-22%.

Recently, most often, treatment is carried out according to the BEACOPP-14 program with the introduction of colony-stimulating factors in standard doses from days 9 to 13 and the resumption of the course on the 15th day. They also carry out 8 cycles of chemistry, then irradiation, as in the BEACORR - basic mode. The effect of the BEACOPP-14 program is similar to the BEACOPP-escalated program with toxicity equal to the BEACOPP-basic program. But the BEACORR-14 program is shorter than the BEACORR-basic and BEACORR-escalated programs, which gives it an additional advantage.

When radical radiation is used after relapses, complete remissions are observed in 90% of patients using polychemotherapy of any first-line regimen. Long-term remissions occur in 60-70%, which depends on the volume of the lesion.

When diagnosed with Hodgkin's lymphoma, the life expectancy of patients with relapses after polychemotherapy or combined induction chemoradiotherapy depends on the nature of the treatment of the disease and the response to treatment. Such patients are divided into three groups:

  • The first group consists of patients who did not have complete remission after the first treatment program. These are primary resistant patients with a median survival of 1.3 years.
  • The second group consists of patients with remission of 1 year and median survival of 2.6 years.
  • The third group includes patients with remission for more than 1 year and a median survival of 4.3 years.

After using first- and second-line chemotherapy according to the SEP, B-CAV, CEVD, Dexa-BEAM, DHAP regimens, repeated complete remissions (after a primary remission of up to 1 year) are noted in 10-15% of patients and in 50-85% of patients with a primary remission of more than 1 year. However, after repeated treatment according to first- and second-line programs, statistically only 11% of patients were able to live for more than 20 years, and 24% of patients with primary remission for more than 1 year.

After high-dose therapy, the survival rate of patients for 3-5 years with a previous remission of more than 1 year and good general condition is 75%, in patients with remission for less than 1 year - 50%, in resistant patients - 20%.

Prognostic features such as B-symptoms, extranodal lesions at relapse, and duration of remission less than 1 year influence 3-year survival. If they are absent, then 100% of patients can live for three years, the presence of one sign reduces the prognosis to 81%, the presence of two – to 40%, with three – it will be 0%.

Infectious complications influence the lethal toxicity of high-dose therapy. According to various studies, it reaches 0-13%, but with transplantation during the third or subsequent relapse it reaches 25%. It follows from this that the main indication for high-dose chemotherapy is the first early and second relapse. Special equipment and trained medical personnel are also required.

Adequate examination and use of modern treatment regimens, complete remissions have been recorded in 70-80% of elderly patients. But concomitant diseases prevent the full scope of therapy. In such patients, the 5-year relapse-free survival rate is only 43%, with an overall relapse-free course of 60%. Patients with local stages who have received adequate treatment have a 5-year survival prognosis of 90%.

Informative video: recurrence of Hodgkin's lymphoma

Complications and rehabilitation

Despite the high percentage of 5-year survival by stage, existing complications worsen the quality of life and lead to death.

Important to remember! Patients have an increased susceptibility to infections, especially after splenectomy. Therefore, antibacterial therapy is administered when signs of infection appear.

After irradiation of the mediastinal area, post-radiation pulmonitis remains, turning into pneumofibrosis. Fibrosis of lung tissue occurs after irradiation if Bleocin, included in the ABVD and BEACOPP regimens, was used during chemotherapy. Therefore, the total dose of Bleocin is set to no more than 200 mg/m². Corticosteroids and antibiotic therapy are used to treat pneumonia.

Infertility is the most serious complication after chemotherapy according to the MOPP regimen and its analogues (SOPP, LVPP, etc.). Because patients may become irreversibly sterile due to chemotherapy and radiation, sperm/egg cryopreservation and surgical transposition of the ovaries from the planned radiotherapy site prior to treatment are discussed and suggested.

The most serious problem is secondary cancer formation. Even years after primary therapy for Hodgkin's lymphoma, lymphogranulomatosis can lead to the death of the patient. Systemic diseases and leukemia arise from the effects of radiation therapy, solid tumors arise from irradiation of organs and tissues, where they begin to grow. Late complications after treatment can lead to mortality, which reduces 20-year survival rate by 20%. Patients may get sick:

  • thyroid dysfunction;
  • cardiovascular pathology;
  • pulmonitis;
  • dysfunction of the gonads;
  • secondary oncological diseases.

The most dangerous complication is that a recurrent malignant neoplasm develops and amounts to:

  • secondary myeloid leukemia – 2.2%;
  • non-Hodshin lymphoma - 1.8%;
  • solid tumor - 7.5%.

When using polychemotherapy with alkylating agents, the percentage of development of secondary myeloid leukemia will be high, and when using the ABVD regimen, it will be low. Frequent secondary leukemia is observed in patients with relapses of Hodgkin lymphoma and repeated therapy.

Age over 40 years and polychemotherapy with alkylating drugs in combination with large volumes of radiation are additional risk factors for the development of secondary leukemia. The survival rate for treatment of secondary leukemia is several months, since they are difficult to treat.

The peak development of secondary myeloid leukemia in patients with Hodgkin lymphoma occurs at 5-7 years of observation. Solid tumors can appear in the period from 5-7 years to 20. Risk factors are age over 40 years and radical radiation therapy. Secondary tumors can develop in the lungs, stomach, thyroid gland, breast, and melanoma is possible. Smoking with Hodgkin's lymphoma increases the risk of developing recurrent cancer.

Conclusion! Late complications require a constant search for optimal treatment regimens for lymphoma, taking into account prognosis factors. The goal of the search is to reduce the dose of chemotherapy and radiation without losing the expected effect of treatment.

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Definition. Hodgkin's lymphoma is a malignant tumor of lymphoid tissue with a specific granulomatous morphological structure, represented by Reed-Sternberg tumor cells, lymphocytic and histocytic cells.

Epidemiology. Accounts for almost 60% of all lymphomas. The incidence of Hodgkin's lymphoma in Russia is 5.1 per 1,000,000 population per year, in the world 4-6 per 1,000,000. Boys are affected somewhat more often than girls. Children are characterized by two peaks of incidence: at 5-7 and 13-15 years.

Classification WHO 2008, based on morphological, immunological, genetic characteristics, divides Hodgkin lymphoma into 2 forms: nodular type Hodgkin lymphoma with lymphoid predominance and classic Hodgkin lymphoma.

Classic Hodgkin lymphoma is divided into variants: modular sclerosis, mixed cell, lymphoid depletion and lymphocyte-rich Hodgkin lymphoma. The nodular type with lymphoid predominance and classic Hodgkin lymphoma differ in clinical characteristics (age of patients, localization of lesions, prognosis), morphological features, immunophenotyping data, genetic characteristics, including association with the Ebstein-Barr virus. Isolation of individual variants of Hodgkin lymphoma must be carried out when examining the primary lesion before treatment.

1. In the variant of lymphoid predominance, the affected tissue consists mainly of small lymphocytes and epithelioid histiocytes. Diagnostic Reed-Sternberg cells are rare. Most Reed-Sternberg cells have folded, curled, or lobulated nuclei, so-called “puffed corn cells.” Depending on the morphological picture, two subvariants are distinguished: nodular and diffuse. Immunophenotypic assessment of L&H cells simultaneously demonstrates the expression of B-lymphocyte antigens (CD19, CD20, CD22, CD79a), which is determined by their origin from B-lymphocytes. In the nodular subvariant, the tumor substrate is L&H cells, which are B cells of follicular origin and express PAX 5+, CD45+, CD20+, EMA+/-, BCL-6+/-, J-chains +/-. In the classical variant, tumor cells have the PAX 5+, CD45-, CD15+, CD30+, EMA-, BCL-6-, J-chain phenotype. Clinical manifestation is sluggish, usually affecting one group of lymph nodes.
2. Nodular sclerosis is the most common histological variant. Hematoxylin and eosin staining reveals eosinophilic collagen fibers of varying widths surrounded by blue lymphoid nodules. Some cells appear to be surrounded by transparent areas. They are called the lacunar variant of Reed-Sternberg cells and are formed as a result of the process of fixation. Nodular sclerosis is divided into two types I and II (according to the criteria of the British histological grading). Nodular sclerosis type II occurs 2 times less frequently than nodular sclerosis type I. In type II, there are areas depleted of lymphocytes or a large number of Reed-Sternberg giant cells of different shapes. This subvariant is accompanied by a worse prognosis than with type I. This process may involve the lower cervical, supraclavicular and mediastinal lymph nodes. Because of the abundance of collagen, radiographic appearance, especially in the mediastinum, may slowly return to normal even when the patient responds to therapy.
3. Mixed-cell variant - the 2nd most common variant. It is characterized by a large number of Reed-Sternberg cells surrounded by reactive histiocytes, small lymphocytes and small foci of necrosis. IHC studies reveal a significant number of CD15+, CD30+, MiM.1+ tumor cells. Focal or partial lymph node involvement is common.
4. Lymphoid depletion is characterized by a decrease in the number of lymphocytes in the affected lymphoid tissue and foci of necrosis. This variant may present as wasting fever syndrome and is often seen with tissue involvement below the diaphragm and bone marrow infiltration. Etiology and pathogenesis. Various epidemiological and serological studies have shown the relationship between Ebstein-Barr virus and the development of Hodgkin lymphoma. A significant percentage of patients have an increased titer of antibodies to the Ebstein-Barr virus, which indicates the relationship and role of the virus in pathogenesis. This theory is confirmed by the presence of the Ebstein-Barr virus genome in Reed-Sternberg cells. In cases where the disease is associated with the Ebstein-Barr virus, the virus is localized in Reed-Sternberg cells, latent production of the Ebstein-Barr virus is determined, and EBV infection is clonal in nature. Both typical Reed-Sternberg cells and their variations express the EBV1 nuclear antigen; type 2 antigen, capsid antigen, early and membrane antigens are not detected. The percentage of virus-associated cases depends on age, sex, ethnicity, histological type and level of development of the country. Thus, EBV-positive tumor genomes are more common in children under 10 years of age and in children living in developing countries. At the same time, it is not possible to identify the Ebstein-Barr virus as the only factor contributing to the development of Hodgkin lymphoma. The disease can result from a variety of pathological processes, including both viral infection and exposure of a genetically predisposed subject to other carcinogens.

Advances in immunohistology and molecular biology have provided new insights into the nature and clonality of these atypical multinucleated giant cells. The presence of an identical gene rearrangement in 90% of Reed-Sternberg cells and in 100% of lymphohistiocytic cells proves their B-cell nature. IHC studies indicate 2 main immunological phenotypes of malignant cells in Hodgkin lymphoma. The first immunophenotype is characterized by the expression of CD20 and J chains and the absence of CD30 and CD15.

Immunophenotype II is characterized by CD30 expression, frequent CD15 expression, and persistent absence of the J chain. Lymphohistiocytic cells have an immunophenotype I, while Reed-Sternberg cells have a phenotype I. Various combinations of immunophenotypes appear to determine the histological subtype of Hodgkin lymphoma. A characteristic feature of Hodgkin lymphoma is the presence of cytokine-producing cells and cells that are their target.

The clinical picture of Hodgkin's lymphoma is very diverse. The disease can begin against the background of complete well-being, and the patient accidentally discovers enlarged lymph nodes. Most often, the disease begins with an enlargement of the cervical, supraclavicular and, somewhat less frequently, axillary lymph nodes. In 15-20% of cases, the disease begins with enlargement of the mediastinal lymph nodes.

Starting in the lymph nodes of one group or another, the pathological process can spread to almost all organs. Damaged lymph nodes are denser than inflammatory ones. Clinical manifestations of damage to a particular organ contribute to the overall picture of the disease. When hilar lymph nodes are involved, patients may present with a nonproductive cough or other symptoms of tracheal or bronchial compression. The most common (tested in a quarter of cases) localization of extranodal lesions in Hodgkin lymphoma is lung tissue. The primary manifestation in the subphrenic region is rare and occurs in only approximately 3% of cases.

Nonspecific symptoms may include fatigue, anorexia, and mild weight loss. Three specific generalization symptoms correlate with prognosis: unexplained fever greater than 38.0°C, unexplained weight loss of 10% in the previous 6 months, and heavy night sweats. Some researchers believe that sweats are less prognostic than other systemic symptoms. Fever is usually one of the early symptoms of the disease. There is no pathognomonic type of fever for Hodgkin lymphoma, but the most characteristic is an intermittent, undulating type of fever. An increase in temperature is often accompanied by chills, and a decrease in temperature is often accompanied by heavy sweats. Excessive sweating, especially at night, is quite often observed in patients with Hodgkin lymphoma. About a third of patients experience itchy skin.

Just as often as lung tissue, the skeletal system is affected. Flat bones are most often affected - vertebrae, sternum, pelvic bones, ribs, then tubular bones. Specific bone marrow involvement is a rare (about 10%) manifestation in Hodgkin lymphoma.

Classification of Hodgkin's lymphoma by stages:
1.Stage I. Damage to a single regional lymph node or a single localization of the process outside the lymph node.
2. Stage II. Involvement of two or more regional lymph nodes located on one side of the diaphragm. May also include extralymphatic localization of the process.
3. Stage III. Damage to regional lymph nodes or extralymphatic localization of the process on both sides of the diaphragm.
4. Stage IV. Disseminated involvement of one or more non-lymphoid organs or tissues, with or without lymph node involvement.

Lung involvement limited to one lobe or root of the lung, combined with lymphadenopathy on the same side, or unilateral pleural effusion with or without lung involvement, but with hilar lymphadenopathy, are regarded as local extralymphatic spread of the disease.

Damage to the liver and bone marrow is always regarded as diffuse extranodal spread of the disease (stage IV).

In addition to the stage and symptoms of intoxication, to select the tactics and extent of treatment for patients, the German group for the study of Hodgkin's disease recommends using a group of prognostic factors, the so-called “risk factors”, which to a greater or lesser extent determine the prognosis of the disease.

These include:
- extranodal lesion within the limits indicated by the symbol E;
- conglomerates of lymph nodes > 10 cm in diameter;
- expansion of the mediastinal shadow on radiographs by enlarged lymph nodes by more than 1/4 of the diameter of the chest at its widest point;
- massive damage to the spleen (the presence of 5 or more foci or diffuse damage to an enlarged organ);
- damage to 3 or more zones of lymph nodes;
- acceleration of ESR above 30 mm/h in stage B.

Diagnostics
Anamnesis. Complaints of heavy sweats, unreasonable increase in temperature and enlargement of peripheral lymph nodes.

Physical studies. On examination, an increase in peripheral lymph nodes is noted, weakened breathing is possible (with damage to the lungs and the development of atelectasis); rarely, an enlargement of the liver and spleen is detected.

Laboratory and instrumental research. There are no changes in peripheral blood specific to Hodgkin lymphoma. There are: increased ESR, neutrophilic leukocytosis (50% of cases), in the later stages - lymphopenia and anemia. Eosinophilia is observed in approximately 20% of cases, especially in patients with itchy skin. Some autoimmune disorders that occur in Hodgkin lymphoma have also been identified: nephrotic syndrome, autoimmune hemolytic anemia, autoimmune neutropenia, autoimmune thrombocytopenia. In 1-2% of Hodgkin lymphoma, a combination of autoimmune hemolytic anemia and autoimmune thrombocytopenia may occur.

The myelogram in patients with Hodgkin's lymphoma does not have significant deviations from the norm. To identify possible bone marrow damage, it is necessary to perform a histological examination. Patients with Hodgkin's lymphoma often have immune system dysfunction at the time of diagnosis, which may persist long after treatment is completed. The activity of NK cells may decrease, the sensitivity of T-suppressors may increase, and humoral immunity usually decreases during treatment.

Chest X-ray is performed in two projections and reveals enlarged mediastinal lymph nodes, infiltrates in the lungs, and the presence of effusion into the pleural cavity. Ultrasound of the abdominal organs and lymph nodes must be performed both for diagnostic purposes and for dynamic monitoring. Computed tomography of the neck, chest, abdomen and pelvis allows you to accurately verify the presence and nature of the lesion.

Radioisotope diagnostics is used to confirm the presence of bone lesions (with technetium). Scintigraphy with gallium citrate is an objective test for assessing the effectiveness of diagnosing lymph node lesions, as well as a control for the state of remission. To confirm the diagnosis, tissue examination is carried out cytologically (tumor imprints), histologically, immunohistochemically, and in doubtful cases (for differential diagnosis with non-Hodgkin lymphoma) molecular genetically. Differential diagnosis is carried out with non-Hodgkin lymphoma, nasopharyngeal carcinoma, soft tissue sarcoma, thymomegaly, reactive hyperplasia.

Indications for consultation with other specialists: cardiologist, neurologist.

An example of a diagnosis: Hodgkin's lymphoma, modular sclerosis, stage III.

Treatment. The goal is maximum eradication of the tumor.

In the 90s it was shown that when treating prognostically favorable variants of Hodgkin's lymphoma, patient survival did not worsen with a decrease in the number of chemotherapy courses and the dose of Hodgkin's lymphoma. The concept of a favorable option differed in different studies, however, as a rule, this group of patients included patients with localized processes, the absence of B-symptoms and a small tumor volume. For example, patients with lesions of the mediastinal lymph nodes could be included in this category if their maximum increase, detected during an X-ray examination, did not exceed 33% in relation to the size of the thoracic cavity. Several pediatric studies completed during this period, using only chemotherapy in the treatment program for such patients, showed that the relapse-free course was 60-92%. The chemotherapy programs used were MOPP, ABVD, SOPP and their combinations. Patients received from 6 to 12 courses of chemotherapy.

Patients with poor prognostic features include those with B-symptoms, large tumor burden, ectnonodal lesions, and stage III-IV disease. One standard approach is to use non-overlapping MOPP/ABVD chemotherapy regimens for 6 months. In recent years, MOPP has often been replaced by SOPP, since cyclophosphamide has less myelotoxicity and is less carcinogenic than mechlorethamine. After chemotherapy is completed, radiation therapy is administered to the affected areas in doses of 15 to 25 Gy. With this strategy, event-free survival usually ranges from 77 to 87%.

The desire to improve the already satisfactory results of treatment of Hodgkin lymphoma has led to the development of more intensive chemotherapy programs for patients with a poor prognosis of the disease. Escalated BEACOPP is a regimen that is superior in effectiveness to the COPP/ABVD combination and has acceptable toxicity.

Doses of chemotherapy drugs used in BEACORR:
- bleomycin - 10 U/m2 on the 7th day; o eoposide - 200 mg/m2 on day 0.1;
- doxorubicin - 35 mg/m2 on day 0;
- cyclophosphamide - 1,200 mg/m2 on day 0;
- MESNA - 240 mg/m2 intravenously 3 and 6 hours after administration of cyclophosphate
fana;
- vincristine - 2 mg/m2 on the 7th day;
- procarbazine - 100 mg/m2 per os on days 0-6;
- prednisolone - 20 m/m2 per os on days 0-13;
- G-CSF - 5 mcg/kg per day after the 8th day until the leukocyte level increases above 1,000.

Non-drug treatment. Hodgkin's lymphoma is a disease sensitive to radiation therapy. Modern pediatric protocols employ the tactic of using low doses and irradiating only the areas involved. In this case, the dose for program patients, as a rule, ranges from 15 to 20 Gy. Recommended single doses are 1.5-1.8 Gy.

Relapses of the disease. The vast majority of relapses occur within the first 3 years, but late relapses also occur. About 50% of patients can be cured if disease progression occurs one year after the end of primary therapy. If a relapse occurs earlier or there is a second relapse, the prognosis is significantly worse. For these patients, the treatment of choice is high-dose chemotherapy with autologous stem cell transplantation. Chemotherapy regimens that can achieve tumor shrinkage without potentiating cardiac toxicity, peripheral stem cell collection, and combinations are effective.

Allogeneic transplantation is also used to treat relapses of Hodgkin's lymphoma. This type of treatment is accompanied by a lower percentage of disease relapses, apparently due to the presence of a graft-versus-tumor mechanism. However, the mortality rate from the transplantation itself exceeds that of autotransplantation, which ultimately results in identical results.
In recent years, regimens with reduced toxicity and tandem transplantation have begun to be studied, when standard autotransplantation is followed by allogeneic transplantation with non-myeloablative regimens. Approaches using adaptive immunotherapy are also being explored.

Indications for consultation with other specialists: in case of cardiomyopathy caused by anthracyclines, consultation with a cardiologist is indicated; in case of endocrine disorders (amenorrhea, azoospermia, hypothyroidism) occurring after irradiation, consultation with an endocrinologist is necessary.

Approximate periods of incapacity: 4-8 months. intensive care.

Further management. Observation by an oncohematologist once every 2 months. for 2 years, then once every 4 months. (3 years), then once every 6 months. (5 years). Necessary examination: general and biochemical blood test, chest x-ray, ultrasound of the lymph nodes, radioisotope examination of the lymph nodes.

Information for the patient. Protective regime (limit physical activity, avoid physiotherapy, excessive insolation).

Forecast. Favorable. When intensive chemotherapy regimens are used in patients with stages III-IV of the disease, event-free survival is 90-95%.

In conclusion, we emphasize that Hodgkin lymphoma in children is a disease in which recovery is the rule and not the exception, and, therefore, risk-adapted therapy, based on modern diagnostics and knowledge accumulated over decades, should contribute not only to the cure of the maximum number of patients, but and reducing the undesirable consequences of therapy.

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