Hodgkin's disease - what is it? The symptoms and treatment
Lymphoma (Hodgkin's lymphoma second name) - refers to a number of diseases and cancer as a medical condition characterized by the lymphatic system, in which the lymphatic tissue cells can be identified Berezovsky-Sternberg-Reed (scientists who discovered this disease).Diagnosed with the disease in children and in adults. To a greater extent detected chlamydia in adolescent children, as well as common in adults aged 20 to 50 years.
What it is?
Hodgkin's Lymphoma (synonyms: lymphoma, Hodgkin's disease, malignant granuloma) - malignancy of lymphoid tissue, a characteristic feature which is the presence of giant cells Reed - Berezovsky - Sternberg (Eng.) Russian detectable by microscopic examination of affected lymph. nodes.
Causes
Until recently considered chlamydia as a disease of infectious origin. It was believed that it could be the causative agent of tuberculosis bacillus. Less often, this role was prescribed streptococcus, E. coli, Treponema pallidum, and diphtheria bacillus. It is also speculated that the virus etiology of Hodgkin's disease, but this is not confirmed.
At the moment, it found that a tumor (gematosarkomy and leukemias) considered certain disorders of the hematopoietic system and malignant cells Berezovsky - Sternberg are the cause of Hodgkin's disease.
It is also not fully clarified certain life factors that may contribute to the disease. These include lifestyle, bad habits, diet and occupational hazards. Several studies provide data about the possible risk for Hodgkin's disease in persons who had been ill with infectious mononucleosis or skin diseases, working on sewing or woodworking industry, in agriculture, as well as among chemists and doctors.
Reported cases of Hodgkin's disease among several members of the same family or in a team. This gives the assumption of the existence of the role slabovirulentnoy infections of viral etiology and genetic predisposition of the organism, however, the final proof of this yet. Thus, certain and exact cause of Hodgkin's disease has not yet been found.
morbid anatomy
Detection of giant cells Reed - Berezovsky - Sternberg and their mononuclear precursor, Hodgkin's cells in the biopsy sample is required criterion for the diagnosis of Hodgkin's disease. According to many authors, but these cells are tumor.
All other cells and fibrosis is a reflection of the body's immune response to tumor growth. The main cells lymphogranulomatous tissue tend to be small, mature T lymphocytes phenotype CD2, CD3, CD4> CD8, CD5 with a different number of B-lymphocytes. In varying degrees, there are histiocytes, eosinophils, neutrophils, plasma cells and fibrosis.
Accordingly, there are 4 major histological types:
- Option with nodular sclerosis - the most common form, 40-50% of all cases. Usually occurs in young women, often located in the mediastinal lymph nodes and has a good prognosis. Characterized by fibrous strands, which divide the lymphoid tissue in the "nodes". It has two main features: the cells Reed - Berezovsky - Sternberg cells and lacunar. Lacunar cells are large, have many cores or multi-blade one nucleus, cytoplasm of a wide, light, frothy.
- Lymphohistiocytic embodiment - about 15% of Hodgkin's lymphoma. More common in men younger than 35 years, it is found in the early stages and has a good prognosis. Dominated by mature lymphocytes, cells Reed - Berezovsky - Sternberg rare. Option of low malignancy.
- Option with the suppression of lymphoid tissue - the most rare, less than 5% of cases. Clinically corresponds IV stage of the disease. It is more common in elderly patients. Complete absence of biopsy lymphocytes predominate Reed cells - Berezovsky - Sternberg as layers or fibrous strands, or combinations thereof.
- Smeshannokletochny embodiment - about 30% of Hodgkin's lymphoma. The most common option in developing countries, in children, the elderly. More common in men are clinically corresponds to II-III stage of disease with typical symptoms and general tendency to generalization process. Smears features a large polymorphism with a variety of cells Reed - Berezovsky - Sternberg, lymphocytes, plasma cells, eosinophils, fibroblasts.
The incidence of the disease - about 1/25 000 / year, which is about 1% of the figure for all malignancies in the world and about 30% of all malignant lymphomas.
symptoms
The first symptoms that people notice are enlarged lymph nodes. Onset of the disease is characterized by the appearance of enlarged dense formations beneath the skin. They are painless to the touch and may occasionally be reduced in size, but then increase again. A significant increase, and pain in the lymph nodes is observed after the consumption of alcohol.
In some cases, may increase the number of groups of regional lymph nodes:
- Cervical and supraclavicular - 60-80% of cases;
- Mediastinal lymph nodes - 50%.
Along with the local symptoms of the patient is seriously concerned about the general manifestations (symptoms of group B):
- at night Profuse sweating (see. causes of excessive sweating in women and men);
- Uncontrolled weight loss (more than 10% of body weight for 6 months);
- The fever persists for more than one week.
Clinic «B» is characterized by more severe course of the disease and allow you to determine the need for the appointment of intensive therapy.
Among other symptoms characteristic for Hodgkin's disease, is isolated:
- Itching;
- ascites;
- Weakness, fatigue, appetite deterioration;
- Bone pain;
- Cough, chest pain, shortness of breath;
- Abdominal pain, indigestion.
In some cases, the only symptom of Hodgkin's disease for a long time is the only constant feeling of fatigue.
Breathing problems occur with an increase in intrathoracic lymph nodes. With the growth of sites they gradually compress the trachea and cause a persistent cough and other respiratory disorders. These symptoms are worse in the supine position. In some cases, patients reported pain in the sternum.
Stage Hodgkin's disease
Clinical manifestations granulomatosis increasing gradually and tested in step 4 (depending on the process prevalence and severity of symptoms).
Stage 1 - swelling in the lymph nodes is one area (I) or in one body outside the lymph nodes.
2 stage - the lymph nodes in two or more areas on one side of the diaphragm (top, bottom) (II) or organ and lymph nodes on one side of the diaphragm (IIE).
Stage 3 - lymph nodes on both sides of the diaphragm (III), or not soprovozhdayushihsya organ lesions (IIIE), or spleen lesion (IIIS), or all together.
- Step III (1) - process localized tumor in the upper abdomen.
- Stage III (2) - lymph nodes in the pelvis and along the aorta.
Stage 4 - the disease spreads in addition to lymph nodes on the internal organs: liver, kidney, intestine, bone marrow and others. with their diffuse disease
To clarify the arrangement used letters E, S and X, below their value. Each stage is divided into category A and B below.
Letter A - the lack of disease in the patient's symptoms
In the letter - the presence of one or more of the following:
- unexplained weight loss of more than 10% of the original in the last 6 months,
- unexplained fever (t> 38 ° C),
- heavy sweats.
The E - tumor spreads to organs and tissue adjacent to the struck large groups of lymph nodes.
Letter S - the defeat of the spleen.
Letter X - three-dimensional formation of a large size.
Diagnostics
To identify malignant granuloma today uses the most modern methods of laboratory and instrumental examination. based:
- on a detailed analysis of the blood;
- highly specific test monitoring the level of tumor markers;
- PET studies;
- MRI study of abdominal organs, chest and neck;
- X-rays;
- Ultrasound studies of lymph nodes and pelvic peritoneum.
The morphological status of the tumor revealed by punctuation lymph nodes, either by complete removal of the node to identify dual large cells (Reed-Sternberg-Berezovsky). With the help of bone marrow study (after biopsy) is carried out differential diagnosis, excluding other malignancies.
Perhaps the appointment of cytogenetic and molecular genetic tests.
How to treat Hodgkin's disease?
The main method of treatment of patients with Hodgkin's disease - combined chemoradiotherapy, which differ in intensity depending on the volume of tumor mass, i.e. the total number of tumor cells in all affected bodies.
In addition, the forecast for the following factors:
- massive defeat of the mediastinum;
- diffuse infiltration and enlargement of the spleen or that it contains more than 5 lesions;
- tissue damage outside of the lymph nodes;
- lymph nodes in three or more areas;
- greater increase in ESR 50 mm / h in step A and greater than 30 mm / h in step B.
For the treatment of patients with a favorable prognosis initially use from 2 to 4 courses of chemotherapy combined with radiation only lymph nodes. In the group with an intermediate prognosis applies 4-6 cycles of chemotherapy and radiation affected areas of lymph nodes. In patients with a poor prognosis of the disease is carried out 8 courses of chemotherapy and radiation zones with a large array of involved lymph nodes.
Forecast
The highest value in the prediction with chlamydia has the disease stage. Patients with stage 4 disease marked 75% five-year survival for patients with stage 2.1 - 95%. Prognostic signs of intoxication is poor. Early signs of unfavorable course of the disease are the "biological" activity indicators.
The biological indicators of activity are:
- alpha-2-globulin more than 10g / l,
- haptoglobin more than 1.5 mg%;
- an increase in the overall analysis of blood ESR 30 mm / h,
- increasing fibrinogen concentrations greater than 5 g / l,
- tserruloplazmina than 0.4 extinction units.
If at least 2 of these 5 parameters exceeds these levels, the stated biological activity of the process.
prevention
Unfortunately, to date, effective prevention of the disease has not been developed. The more attention is paid to the prevention of relapse, this requires strict compliance program prescribed treatment of Hodgkin's disease and the implementation of the desired mode and rhythm of everyday life.
Among the most common causes of repeated manifestations of the disease can be called insolation, pregnancy. After transferring the disease the possibility of pregnancy is permitted after two years from the date of the remission.
