Mar 05, 2018
Lymphogranulomatosis is a primary tumor disease of the lymphatic system, accompanied by an increase in lymph nodes, often spleen and other organs. When microscopic examination of the affected tissue, granulomas containing Berezovsky-Sternberg-Read cells are found. For the first time the disease was described by the English doctor T. Hodzhkin in 1832.23 years later, S. Wilks called this condition Hodgkin's disease, having studied the cases described by Hodgkin and adding 11 own observations to them. This disease occurs only in humans and more often affects representatives of the European race. Lymphogranulomatosis can occur at any age. However, there are two peak incidence rates: between the ages of 20-29 and over 55.Both men and women, with the exception of children under 10 years of age( often sick with boys), suffer from lymphogranulomatosis equally often, but men are still somewhat more likely.
Symptoms of the disease are very diverse. More often, the first symptoms of the disease include an increase in lymph nodes, primarily the cervico-supraclavicular region, mainly on the right. Lymph nodes are mobile, dense, elastic, not soldered to the skin, only in rare cases are painful. In 1/5 of patients, lymph nodes of the mediastinum primarily increase, which can be accidentally detected in fluorography. In a number of patients, the disease can initially occur with fever, night sweats, rapid weight loss. Fever is an important symptom of other forms of the disease, while prolonged periods of normal temperature can be replaced by a periodic increase in it.
Itching is characteristic of the skin, sometimes intolerable. In the blood there is a moderate neutrophilic leukocytosis, occasionally eosinophilia increased by ESR.
When the disease progresses, anemia and cachexia develop. The disease is characterized by a variety of manifestations, which is associated with the possibility of proliferation of lymphoid tissue in any of the internal organs.
The diagnosis can be refined by biopsy of the lymph node, in which the characteristic multinucleated giant cells of Berezovsky-Sternberg are found. With a long enough flow, a serious complication of lymphogranulomatosis is a renal mercury, which can lead to chronic uremia.
According to the international clinical classification, four stages are distinguished according to the degree of prevalence of the process:
I stage( local forms) - affection of one or two adjacent groups of lymph nodes or one extralimphatic organ;
II stage( regional forms) - the defeat of any groups of lymph nodes on one side of the diaphragm, which can be combined with the localized involvement of one extralimphatic organ;
III stage( generalized( common) forms) - lymph node involvement on both sides of the diaphragm, which can be accompanied by localized involvement of one of the extra-lymphatic organs, the spleen;
IV stage( disseminated forms) - involvement in the process of bone marrow, pulmonary parenchyma, pleura, liver, bone tissue, gastrointestinal tract in combination with affection of lymph nodes and spleen.
Each stage, in turn, is divided depending on the absence( A) or the presence( B) of one or more symptoms of intoxication: elevated body temperature above 37 ° C, night sweats;dermal itching, rapid weight loss by more than 10%( with the presence of only pruritus itch is considered insufficient for referring the case to subgroup B).
General and biochemical blood tests
Biopsy of the affected lymph node with morphological and immunological examination.
The main analysis allowing to confirm the diagnosis of lymphogranulomatosis is a microscopic examination of a sample of tumor lymphoid tissue obtained by biopsy. This tissue is sent to a morphological study under a microscope to determine whether a lymph node is actually filled with tumor cells and whether there are specific Berezovsky-Sternberg-Read cells present in it. To confirm the diagnosis, an immunohistochemical study can be performed to determine the immunological characteristics of the tumor cells.
Radiodiagnosis( radiography, computed tomography, magnetic resonance imaging) makes it possible to detect the presence of tumor formations in various parts of the body that are not available to the physician during external examination.
Methods of radiation diagnosis are used to determine the stage of lymphogranulomatosis.
There are 5 types of lymphogranulomatosis. The correct diagnosis can be made only after examination of the tissue of the lymph node, obtained by biopsy.
Variants of nodular sclerosis( the most common is 75%)
Mixed cell variant
Option with lymphoid depletion( least common - less than 5%)
Variant with a large number of lymphocytes
Nodular variant with a predominance of lymphocytes
Currently, the committeeWHO for all countries identified the most effective regimens for the therapy of lymphogranulomatosis. Arbitrary modification of these approved chemotherapy regimens is unacceptable.
At the I-II stage of the disease, surgical removal of all lymph nodes of the affected area or fractionated irradiation with their gamma rays in the total dose of 3500-4500 rad is applied, which is equally effective: there are no relapses of the disease in this zone. It is obligatory to conduct preventive irradiation of adjacent areas of the body to eliminate micrometastases in them. Such therapy is called radical. Radiation treatment is carried out in specialized hospitals, using figured shielding of vital organs, which allows to increase the absorbed dose of radiation in the outbreak. At present, irradiation from large fields in the form of a mantle is more often used. In some cases, they resort to total total irradiation with gamma rays in doses of 100-200 rad.
In generalized and disseminated stages( III-IV), polychemotherapy is indicated. The most effective 2-week cycle scheme proposed in 1967 Bernard: embichine( Mustargen) - 6-10 mg intravenously simultaneously with vincristine - 1,5-2 mg, only in the 1st and 8th days of the cycle;Natulan( procarbazine). - 50-150 mg per day and prednisolone - 40 mg per day followed by a 2-week break. Carry at least 6 of these cycles for six months. This scheme in approximately 80% of cases provides a complete remission of 5 years, even in the III-IV stage of lymphogranulomatosis. Instead of embihin, cyclophosphamide( 800-1000 mg intravenously) is sometimes used on the same days. Cytostatics known in the past - dipin and degranol - are almost never used.
Very effective and combined treatment: first 2-4 courses of polychemotherapy, then radiotherapy for a radical program. In the III and IV stages of granulomatosis after the described therapy, maintenance treatment with vinblastine is continued for 2-3 years, which makes it possible to halve the percentage of relapses. It is these schemes that have made it possible to achieve a nearly complete cure of dozens of patients with lymphogranulomatosis.
After reaching remission, the patient should remain under the supervision of a doctor, since it is important to diagnose the possibility of relapse in time.
Cytostatic drugs, like radiation therapy, cause side reactions: leukopenia, thrombocytopenia, reversible hair loss on the head, dyspeptic and dysuric phenomena. For outpatient use of these drugs, blood testing is mandatory 2 times a week.
When the leukocyte count drops to 3,000-2,000 in 1 μl, cyclophosphamide and natulane dosages are reduced by a factor of 2-4, with leukopenia less than 2000 in 1 μl or thrombocytopenia below 50,000 in 1 μl of cytostatics are canceled and the patient is hospitalized.
In a complex of medical measures, antibiotics, cardiovascular drugs, blood transfusion, etc. are used. At the beginning of the disease, butadion is successfully used to lower the temperature.
The choice of method of treatment is determined by the stage of the process. To exclude the defeat of the internal organs and lymph nodes perform lymphography( the introduction of radiopaque substances into the lymphatic vessels of the limbs), often resort to diagnostic laparotomy with liver biopsy and peritoneal lymph nodes. The spleen is removed as an important focus of the disease.
Pregnancy in a patient with lymphogranulomatosis women should be discontinued only in case of an exacerbation of the disease. In our country and abroad, dozens of cases have been described, when women who received radiation, medicinal or combined treatment for lymphogranulomatosis, became pregnant and gave birth to healthy children. However, in all cases, the doctor decides whether to maintain the pregnancy.
The prognosis depends on the features of the course of lymphogranulomatosis, the age of the patient, the clinical stage of the disease, the histological type, etc. In acute and subacute disease, the prognosis is unfavorable: patients die usually within 1-3 months.up to 1 year. In chronic lymphogranulomatosis, the prognosis is relatively favorable. The disease can last for a long time( up to 15 years with continuously recurrent flow, in other cases and longer).In 40% of all patients, especially in the I-II stages and favorable prognostic factors, there are no relapses for 10 years or more. The ability to work in the case of prolonged remissions is not violated.
Prevention is aimed at preventing relapses. Patients with lymphogranulomatosis are subject to follow-up oncologist. In a survey that should be carried out every 6 months in the first three years, then once a year it is necessary to pay attention to biological signs of activity, which are often the first symptoms of relapse( an increase in POP more than 30 mm / h, an increase in the level of a2-globulins and fibrinogen).Patients with lymphogranulomatosis are counter-indicative of overheating, direct insolation, thermal physiotherapy. An increase in the number of relapses due to pregnancy has been noted.
Prevention of lymphogranulomatosis consists in reducing the effect of mutagens on the body( chemicals, radiation, UV rays).Of great importance are sanation of infectious foci, hardening. Electrotherapy and some other methods of physiotherapy in the elderly should be limited to the extent possible.
Periodic medical examinations of workers are of great importance, in particular, fluorography helps identify early, preclinical stages of lymphogranulomatosis with mediastinal nodes.