Lymphogranulomatosis (Hodgkin's disease)
Lymphogranulomatosis is a malignant disease manifested by hyperplasia of cells of the lymphoreticular system. It is characterized by primary generalization (prevalence throughout the body) and systemicity of the process. A distinctive morphological feature of the process is the formation of granulomas, which contain Berezovsky-Sternberg cells and include several nuclei of their predecessors - Hodgkin cells.
Incidence varies between 1.8-3.2 cases per year per 100,000 population. Mostly men are sick (2 times more often than women). Of the age groups, people 16-30 and over 60 years old are more prone to the disease. In the incidence structure of Hodgkin's disease, the share of children accounts for about 15%. The causes of the disease have not been clarified, which is evidenced by the presence of many theories. The main ones include hereditary, viral and immune, each of which does not provide comprehensive answers to many questions.
Symptoms of lymphogranulomatosis
The basis of the development of the process is the formation of granulomas in nodes of the lymphatic system and organs of the body with the growth of fibrous structures. Symptoms of lymphogranulomatosis differ in wide polymorphism (differences in the clinical picture), which is due to:
- morphological (histological) variety of lymphogranulomatosis;
- participation in the process of lymph nodes of different localization and different internal organs.
The first signs of Hodgkin's disease are non-specific: weakness, intoxication, sweating, wave-like fever with temperature rises up to 38°C, persistent moderate/generalized itching of the skin, weight loss of 10-15% in 6 months.
In 20-30% of patients, the first manifestation is an increase in lymph nodes (axillary, cervical, inguinal). At the beginning of the disease, the lymph nodes are not fused, mobile, elastic and practically painless. Later, their size increases, they merge and form painful conglomerates. The skin over the enlarged lymph nodes becomes flaky, it becomes hyperemic and stretched. Further, the process spreads through the lymph/blood stream to various organs with a developed capillary network: liver, spleen, lungs, bones, which determines the later symptoms of Hodgkin's lymphoma.
When the process moves to the mediastinal lymph nodes, patients develop swallowing disorders, shortness of breath, and a dry cough. In cases of involvement of the retroperitoneal/mesenteric lymph nodes in the process, patients note a pronounced abdominal pain syndrome, swelling of the lower extremities. Lung tissues are affected in 25% of cases.
Symptoms of lymphogranulomatosis of the lungs are similar to the symptoms of pneumonia (weakened breathing, wheezing, strong cough, viscous sputum with mucous-purulent content). In the preclinical stages, the disease is most often diagnosed during radiography. When the process moves to the pleura, the clinic of pleurisy develops.
When the bones are affected, the pelvic and tubular bones, sternum, vertebral column and ribs are mainly affected. The process is accompanied by complaints of vertebra and paravertebral pain (pain along the axis of the spine), destruction of the vertebral bodies occurs. Infiltration into the bone marrow causes various abnormalities on the part of the blood (anemia, leuko/thrombocytopenia). When the process moves to the spinal cord, paralysis is noted.
Symptoms of the abdominal and generalized form of Hodgkin's disease are caused by the involvement of the spleen and liver, which is accompanied by:
- increasing their sizes;
- a decrease in the content of protein fractions in the blood;
- increasing the content of alkaline phosphatase.
Lymphogranulomatosis of the gastrointestinal tract is accompanied by intestinal bleeding, violations of the integrity of the mucous membrane up to intestinal perforation with the development of peritonitis. With the generalized form in the late stages, almost all organs are involved in the process (thyroid and mammary glands, tonsils, ovaries, skin, heart).
The course of the process is largely determined by the histological structure of lymphogranulomatosis:
- with the lymphohistiocytic variant – a relatively benign and long course (up to 15 years);
- with mixed cellularity - duration up to 4 years.
Lymphoid depletion proceeds subacutely, and the nodular form is characterized by late symptoms and the involvement of mediastinal lymphoid tissue in the process.
Stages of lymphogranulomatosis
The classification is based on the criteria of prevalence of the malignant process.
Hodgkin's lymphoma (stage 1) – only one local group of lymph nodes or only one organ is affected.
Hodgkin's lymphoma (stage 2) – more than 2 areas of lymph nodes of the diaphragm are involved, or any organ with adjacent lymph nodes.
Hodgkin's lymphoma (stage 3) is a combined lesion of the lymph nodes on both sides of the diaphragm, or organ.
Hodgkin's lymphoma (stage 4) – lymph nodes and several organs are involved.
Correct staging of lymphogranulomatosis is possible only after obtaining positive histological results. The stage of the disease is confirmed by the detection of Berezovsky-Sternberg cells in the biopsy of peripheral lymph nodes.
Methods of treatment of lymphogranulomatosis
The latest advances in medicine and approaches to the treatment of lymphogranulomatosis are based on the potential for a complete cure for Hodgkin's lymphoma. For this purpose, various schemes of irradiation, polychemotherapy and their combinations are carried out.
Radiation therapy, as a monomethod, is carried out at the I-IIA stage in the form of subtotal/total irradiation of all groups of lymph nodes. In this case, both affected lymph nodes and "healthy" ones are subject to radiation for the purpose of prevention. In some cases, preliminary surgical removal of individual/groups of lymph nodes and spleen is performed.
Combined methods of treatment of Hodgkin's disease are used in stages IIB - IIIA:
- initial polychemotherapy with parallel irradiation on minimal modes of exclusively enlarged lymph nodes;
- at the second stage – irradiation with maximum doses of other lymph nodes and the appointment of drugs that support polychemotherapy for 1-2 years.
In the case of a disseminated process of lymphogranulomatosis (stages III and IV), long-term cyclic induction polychemotherapy is carried out using various methods (CVPP, MORR, SURR, ABVD, SORR). To maintain and extend periods of remission, cycles of drug treatment or radical irradiation are periodically carried out.
Surgical methods have no independent value in the treatment of Hodgkin's lymphoma. At the end of the therapy (treatment cycle), its effectiveness is evaluated. The result can be:
- full recovery;
- partial remission;
- clinical improvement – weak dynamics of the process;
- lack of positive dynamics - the process continues or progresses.
Median 5-year survival for stages 1-2 after treatment is about 90%, for stages 3A-3B 80-60%, and for the final stage - less than 40%.
Prevention
Given the lack of knowledge about the causes and risk factors of the disease, there is no specific prevention of Hodgkin's disease. The following are recommended as preventive measures:
- avoid contact with potentially dangerous substances that include carcinogens;
- lead a healthy lifestyle;
- follow the principles of a healthy diet and maintain your immunity.
Due to the fact that there are no measures to prevent Hodgkin's disease, the timeliness of the diagnosis of Hodgkin's lymphoma and the timely initiation of treatment come to the fore. Therefore, at the appearance of the first signs and suspicion of the disease, an urgent visit to an oncologist is necessary. In this regard, it is extremely important to regularly undergo preventive fluorography, since in some cases the diagnosis of lymphogranulomatosis at the preclinical stage is carried out precisely during such examinations.
In case of non-compliance with the regime and terms of maintenance therapy, relapses of the disease may occur. They can also provoke pregnancy and heavy loads. All patients with Hodgkin's disease must be registered at a dispensary and be observed by an oncologist.
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