Hodgkin's lymphoma - a tumor disease of the lymphatic system. Hodgkin's disease occurs 3 times more often in families that already have been reported, these patients, compared with families where they were not.
Causes of Hodgkin's disease finally clarified. Some experts believe that the lymphoma associated with Epstein-Barr virus.
manifestation of Hodgkin's disease
Symptoms of Hodgkin's disease are very diverse. Beginning in the lymph nodes, painful process can be extended to almost all organs, accompanied by different manifestations of severe intoxication (weakness, lethargy, drowsiness, headache).
The primary lesion of an organ or system, and determines the picture of the disease.
Hodgkin's disease is usually the first manifestation becomes enlarged lymph nodes; in 60-75% of cases, the process begins in the cervical-supraclavicular lymph nodes, more often on the right. Typically, lymph node enlargement is not accompanied by breach of health of the patient. Enlarged lymph nodes are movable, not soldered to the skin, in rare cases, painful. Gradually, sometimes rapidly increasing, they merge into larger units. Some patients have pain in the enlarged lymph nodes after drinking alcohol.
In some patients the disease begins to increase in mediastinal lymph nodes. This increase may be accidentally discovered when x-rays or occur in the later stages, when the dimensions of education are significant, cough, shortness of breath, rarely - pain behind the breastbone.
In rare cases, the disease begins with an isolated lesion okoloaortalnyh lymph nodes. The patient complains of pain in the lumbar region, occurring mainly at night.
Sometimes the disease begins acutely with fever, night sweats, rapid weight loss. Usually in these cases, a slight increase in the lymph nodes appears later.
The most frequent localization of Hodgkin's disease is a lung tissue. Lung Lesions usually are not accompanied by appearances. Quite often with chlamydia is detected accumulation of fluid in the pleural cavity. Typically, this is a sign of a specific lesion pleura, sometimes visible under X-ray examination.
pleural involvement occurs commonly in patients with Hodgkin's disease with enlarged mediastinal lymph nodes or foci in the lung tissue. The tumor in the lymph nodes of the mediastinum may grow in the heart, esophagus, trachea.
Skeletal system - as frequent as lung tissue, localization of the disease in all cases of the disease. Often affects the vertebrae, then the sternum, pelvis, ribs, at least - the long bones. Involvement in the process of bone manifested by pain, radiological diagnosis is usually delayed. In rare cases, bone loss (sternum) may be the first visible sign of Hodgkin's disease.
liver damage due to the large compensatory capacity of the body are detected late. Specific characteristic features of liver damage is not present.
Gastrointestinal usually suffers again in connection with compression or tumor invasion of the affected lymph nodes. However, in some cases, it found lymphogranulomatous defeat of the stomach and small intestine. The process generally involves the submucosal layer is not formed ulcers.
Sometimes there are lesions of the central nervous system, primarily the spinal cord, they give a serious neurological disorder.
Quite often with chlamydia variety of skin changes: scratches, allergic reactions, dry.
Greater or lesser sweating celebrated almost all patients. Heavy night sweats, underwear makes me often accompany periods of fever and indicate severe illness.
Itching is approximately one third of patients. Its severity varies widely: from mild itching in the areas of enlarged lymph nodes before spreading dermatitis scratching all over. This itching is very painful for the patient, depriving him of sleep, appetite, leads to mental disorders. Finally, weight loss accompanied by severe exacerbations and terminal stages of the disease.
Diagnostics
Even with enough convincing clinical picture only histological examination allows detecting lymphogranuloma definitively confirm the diagnosis. Pathological diagnosis can be considered valid only if an histological variant Berezovsky-Sternberg cells.
Histological analysis not only confirms and establishes the disease, but also determines the morphological variant. Pathological diagnosis of Hodgkin's disease is considered to be beyond doubt, if it is confirmed the three morphology. Sometimes getting the material for histological examination difficult location of the lesions in the lymph nodes of the mediastinum or retroperitoneal space.
For the diagnosis of diseases caused by increased only mediastinal lymph nodes, apply diagnostic opening the chest cavity.
Localization of Hodgkin's disease only in retroperitoneal nodes is extremely rare, but in such cases, require histological confirmation of the diagnosis, that is, it shows the opening of the abdominal diagnostic cavity.
Involvement of mediastinal lymph nodes, lung roots, lung tissue, pleura, bone, is detected by X-ray studies, including computerized tomography. To study the para-aortic lymph nodes are used lymphography.
scan method of retroperitoneal lymph nodes is not accurate enough (percentage of false positive and false negative responses reaches 30-35%). The best method is a direct contrast lymphography (error method 17-30%). A refinement step is performed additional disease research methods, which include:
- medical examination
- chest X-ray
- percutaneous biopsy of bone marrow
- scanning liver, spleen, and radionuclide scans
- contrast angiography
Hodgkin's disease treatment
Modern methods of treatment of Hodgkin's disease is based on the concept of curability of the disease. While Hodgkin local lesion remains more groups of lymph nodes (step 1-2), it can be cured by irradiation. The results of prolonged use of chemotherapy to the limit of healthy tissue tolerability suggest cure and distributed process.
Radical radiotherapy, ie radiotherapy at the beginning of the disease at a dose of 35-45 Gy to focus on sufficient space (wide field, including all groups lymph nodes and road drainage), with a sufficiently high energy beam (megavoltage therapy), capable of fully cured 90% of patients with limited forms of disease. Exceptions are patients with stage 1-2 in which the mediastinal lymph nodes more than 1/3 the diameter of the thorax. These patients should receive additional chemotherapy.
Chemotherapy is administered in the time of diagnosis. Also using radiotherapy. Many hematologists believe that it is necessary to combine chemotherapy and radiation therapy.
Proper treatment of the first step can lead to complete recovery. Chemotherapy and radiation of all groups of lymph nodes are very toxic. Patients tolerate treatment difficult due to frequent adverse reactions, including nausea and vomiting; hypothyroidism, Infertility, secondary lesions of the bone marrow, including acute leukemia.
Hodgkin's disease treatment regimens
- MOTAPMs - Mustag, onkovir (vincristine), procarbazine, prednisone. Apply at least for 6 cycles plus 2 additional cycles after reaching complete remission.
- ABVD - Adriamycin (doksrubitsin), bleomycin, vinblastine, dacarbazine. This scheme is highly effective in patients with relapses. In combination chemotherapy often used ABVD scheme.
- IWLP (similar to the MOPP regimen, Oncovin replaced vinblastine at a dose of 6 mg / m2).
If therapy is not effective or within one year after remission relapse the patients underwent more powerful therapy - DexaBEAM: where Deha - dexamethasone In - BCNU, E - etapizid, A - Aracena (Cytosar), M melfolan. Spend 2 courses. If you get the effect, then spend the bone marrow collection or blood stem cells, and make such a patient autologous transplantation. Otherwise, there is a bad outcome.
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 "biological" activity indicators. The biological indicators of activity are:
- an increase in the overall analysis of blood ESR more than 30 mm / h,
- increase the concentration of fibrinogen more than 5 g / l,
- alpha-2-globulin more than 10g / l,
- haptoglobin more than 1.5 mg%;
- tserruloplazmina than 0.4 extinction units.
If at least 2 of these 5 parameters exceeds these levels, the stated biological activity of the process.
