Lymphogranulomatosis (Hodgkin's lymphoma) is a malignant change in lymphoid tissue. It manifests itself in the form of an increase in lymph nodes, most often the supraclavicular, mandibular or axillary nodes are affected. The process begins in one of the lymph nodes and then spreads throughout the body. The disease is successfully treated, the patient is constantly monitored after a course of therapy, since the disease tends to return.
What is lymphogranulomatosis?
The disease begins with the simultaneous appearance of granulomas and Berezovsky-Sternberg cells in any one lymph node. The formation is considered by the immune system as an aggressive foreign agent and is attacked by white blood cells. Lymphocytes, erythrocytes, eosinophils and other cells are sent to neutralize and remove a foreign formation, designed to protect the internal environment of the body.
They form a dense cell barrier around the affected node. The whole structure, condensing, forms into a granuloma, in which inflammatory processes occur, gradually increasing the size of the node - this is lymphogranulomatosis.
Symptoms continue to increase as the disease develops. Altered cell clones migrate through the lymph node system, as well as into adjacent organs and tissues. Settling in new areas, pathological cells provoke a wave of growth of new granulomas. Gradually overgrown malignant cells replace healthy tissue, which leads to disruption of organs.
The patient has an increase in the spleen, weight loss, general weakness. At the present stage, medicine has an accurate idea of the methods of treatment, but the causes and prerequisites for the occurrence of pathology remain unclear.
Cancer or not?
From a medical point of view, cancer is a mutation of epithelial tissue in which the affected cells grow in the lumen of the internal organs. The tissue of the lymph nodes is not epithelial, therefore, from a scientific point of view, lymphogranulomatosis is not an oncology in the literal sense. But there are common signs that combine cancer and lymphogranulomatosis.
Symptoms and general features:
- Infiltrating (malignant) cell growth, germination in neighboring organs and tissues (metastases).
- Intoxication of the body, exhaustion of the patient.
- The same treatment principle is the destruction of mutated cells by chemotherapy and radiation therapy.
Patients, at the level of communication with specialists, call lymphogranulomatosis one of the forms of cancer, and this does not cause opposition or objection from doctors.
Causes of occurrence
Today, medicine can diagnose lymphogranulomatosis with high accuracy. Symptoms and the clinical picture of the disease are known to doctors, but the causes of the onset of the disease, like all cancer, are not known reliably. According to long-term statistics, people of two age categories are most susceptible to Hodgkin's lymphoma: the first includes men and women from 15 to 30 years old, and the second in the bulk are men over 50 years old.
Numerous studies on the occurrence of the disease have so far provided little information. Most specialists are inclined to believe that infections, heredity or malfunctions of the immune system serve as a trigger for cell change. But there is no definite answer about the causes of the onset of the disease.
Distribution mechanism
The basic difference between lymphogranulomatosis and other types of lymphomas is as follows:
- The presence of double-core giant Reed-Sternberg cells in the nodes.
- The presence of mononuclear large Hodgkin cells.
- The inclusion of a large number of blood cells in the lymphoma (red blood cells, white blood cells, eosinophils, plasmocytes, etc.).
There is another characteristic that defines lymphogranulomatosis. Symptoms occur when the first lymph node is damaged (on the neck, in the subclavian region, in the mediastinum), and metastases spread through the lymphatic and blood vessels, sprouting into organs adjacent to the foci - lungs, gastrointestinal tract, bone marrow, kidneys, etc.
Classification and stages of the disease
Specialists distinguish two forms of the disease:
- Local - lymph nodes of one group are affected. Allocate forms of Hodgkin's disease - peripheral, pulmonary, skin, mediastinal, abdominal, nervous, etc.
- Generalized - metastases penetrate the spleen, kidneys, stomach, liver, skin.
Lymphogranulomatosis can occur both in acute form and in a chronic course. The clinical classification of the disease is determined by four stages of development:
- The first stage - the lesion affected one group of lymph nodes or one extralymphatic organ.
- The second stage - two or more groups of lymph nodes located on one of the sides of the diaphragm, or one extra-lymphatic organ together with regional lymph nodes are affected.
- The third stage of the disease - the lymph nodes on both sides of the diaphragm are affected, one extra-lymphatic organ or spleen may also be affected, or the lesion comprehends them comprehensively.
- The fourth stage - the disease affects one or more internal organs (bone marrow, spleen, lungs, gastrointestinal tract, etc.), while the lymph nodes may or may not be involved in the disease.
Symptoms of the disease: swollen lymph nodes
At the initial stage, no one can diagnose lymphogranulomatosis. Symptoms in adults and children do not give a clinical picture of the disease. Sometimes this happens by chance, with ultrasound examination of the lungs, in this case, enlarged structures of the nodes will be visible on the image. Only in the later stages, with obvious manifestations of the pathology, is a conclusion about the disease made.
Manifestations of Hodgkin's disease:
- Enlarged lymph nodes in size.
- Systemic manifestations of the disease.
- Lesions of internal organs and severe symptoms of malfunction processes in their functionality.
The first and constant sign of the disease is an enlargement of one or more lymph nodes. The manifestation can occur anywhere - in the armpit, on the neck, in the groin. The patient does not feel any discomfort - there is no fever, the general health is normal. Nodules during palpation do not cause pain, they roll under the skin, resembling a dense ball, which gradually increases in size.
Lymphogranulomatosis in children
Children are also prone to Hodgkin's lymphoma (lymphogranulomatosis). Symptoms in children do not differ from the picture of the disease in adults, but with some additional manifestations:
- Increased sweating, especially at night.
- Apathy, lethargy, decreased muscle tone.
- Headaches, palpitations (tachycardia).
- Stiffness in the movements.
- Anemia, weight loss.
At the last stage of the disease, "children's lymphogranulomatosis" symptoms, the clinic of its manifestations do not differ from the same condition in adults.
Disease development
After the defeat of one of the nodes, the next stage is the spread of the disease of the lymph nodes from the cervical to the chest, pelvic organs and lower limbs. Deterioration of well-being begins at the moment when the swollen nodes begin to compress the organs adjacent to them, which leads to the appearance of the following manifestations:
- Cough - is the result of compression of the bronchi and irritation of the receptors. It is not treated with antitussive drugs.
- Shortness of breath - occurs due to compression of lung tissue, trachea or bronchi. The patient may lack air during a period of intensive training, with an overgrown pathology and at rest.
- Violation of swallowing. Increasing in size, intrathoracic lymph nodes squeeze the esophagus. As a result, it is difficult for a person to swallow while taking solid food, and later liquid.
- Disorders in the digestive tract - overgrowth of nodes leads to stagnation of food due to squeezing of individual sections of the intestine, which leads to bloating, diarrhea, constipation, etc. Tissue necrosis can also occur due to squeezing of blood vessels.
- Kidney dysfunction - occur due to damage to the lumbar lymph nodes, putting pressure on the kidney tissue. Since the kidneys are a paired organ, when acting on one of them, the patient will not feel changes, with a bilateral increase in pressure, renal failure occurs. This situation is extremely rare.
- Swelling. Blood in the heart flows from the superior and inferior vena cava. When squeezed by the enlarged node of the superior vein, swelling of the face, hands, neck occurs, and with pressure on the inferior vein, swelling occurs in the legs and internal organs.
- Disturbances in the nervous system occur as a result of compression of the spinal cord. With damage, the sensitivity and motor activity of the upper or lower extremities are impaired. Disturbances in the functioning of the nervous system are extremely rare and the last diagnosis, which is considered in such cases, is lymphogranulomatosis.
Symptoms of damage to internal organs
Hodgkin's lymphoma, like all tumor processes, grows with metastases in the tissues of any organ. Manifestations of the disease can be expressed in the following:
- An increase in liver size. This symptom is observed in most patients with lymphoma. Malfunctions in the work of the organ begin from the moment when the overgrown lymph nodes displace healthy tissue.
- Spleen enlargement - this phenomenon catches up to 30% of patients with Hodgkin's disease already in the last stages of the disease. The development of pathology does not bring the patient any pain and is asymptomatic.
- Violation of the hematopoiesis process - occurs with the growth of pathological tissue in the cavities of the bones, while the cells of the bone marrow are replaced by sprouted metastases. Pathology can lead to aplastic anemia (decreased production and renewal of blood cells). Pediatric lymphogranulomatosis is also manifested. Symptoms in adults, a blood test and the overall picture are identical.
- Damage to the lungs occurs in 10 or 15% of cases of Hodgkin's disease. Symptoms are manifested during the germination of altered tissue in the lungs. At the first stage, the patient does not feel changes, and at the last stage, respiratory failure, shortness of breath, intense dry cough, etc.
- Bone tissue disorder is a severe type of lesion in which, in addition to inhibiting bone marrow activity, bone tissue is disturbed. Tumor cells disrupt bone structures, complaints of pain are received, as a result of the slightest effort, pathological fractures occur. The most common lesions are vertebrae, pelvic bones, sternum.
- Itchy skin occurs in a patient due to a significant increase in white blood cells, which, when destroyed, release active substances that irritate the skin.
The above list is the most important and frequent manifestations of the disease, considered in the diagnosis of lymphogranulomatosis. Symptoms of Hodgkin lymphoma can occur in any organ and disrupt its work, structure and functionality.
Diagnostics
The definition of the disease is difficult due to the non-specificity of its manifestations, therefore, in most cases, only at a late stage of development, lymphogranulomatosis is diagnosed. Symptoms, an analysis of the general condition, and even clinical studies give a complete picture only after the detection of tightened lymph nodes. Only one node is extremely rare. At the stage of visual manifestation, usually there are already several foci.
This disease is characterized by a late start of therapy, which sometimes does not lead to a positive outcome. This is the main danger of Hodgkin's disease (lymphogranulomatosis). Symptoms, a blood test and a study of other indicators lead the patient to inpatient treatment in the hematology department.
Diagnostic Methods:
- Bone marrow puncture
- Immunophenotyping of lymphocytes.
- Instrumental examination.
- Histology of the lymph nodes for lymphogranulomatosis (symptoms).
- Blood is examined for biochemistry, and its general analysis is also carried out.
Treatment
Modern medicine successfully treats lymphogranulomatosis. Symptoms in adults, analysis of all manifestations, diagnostic accuracy allow an experienced hematologist to build an effective treatment tactic, which includes:
- Chemotherapy (medication).
- Radiotherapy.
- Surgery
With a timely and correct diagnosis, adequate therapeutic measures in 80% of cases it is possible to achieve a stable remission.
The prognosis of recovery is due to the following factors:
- Stage of the disease. The start of therapy at the 1st and 2nd stages of the development of the disease guarantees 90% of the onset of complete remission after a course of chemotherapy and radiotherapy. The start of treatment at stages 3 and 4 allows us to count on the success of treatment in 80% of cases.
- The defeat of internal organs by metastases often causes irreversible damage, treatment can not restore the structure and function.
- With damage to the structure of the lymph nodes, proper treatment is able to restore their functions partially or completely. With lymphoid depletion, reversible processes do not start. An aggravating circumstance is that the number of lymphocytes in the body will be reduced.
- Only 2-5% of cases of lymphogranulomatosis are resistant to any type of therapy.
- Relapses are observed in 10-30% of patients who underwent the entire course of chemo- and radiotherapy. The return of the disease is possible within a few months or years after the end of treatment.