Lymphogranulomatosis or Hodgkin's disease is a disease that causes damage to the human lymphatic system.
During its development, the formation of specific polymorphic granulomas in different internal organs (lymph nodes, spleen, etc.) occurs. Pathology is named after the scientist who first recorded and described in detail its symptoms. Lymphogranulomatosis is also called Hodgkin's disease. An alternative designation is Hodgkin's lymphoma.
The disease is most susceptible to young people - from 20 years. More rarely, pathology is detected in patients older than 60 years of age. The disease is 1.5 - 2 times more often affects the male. Lymphogranulomatosis is the 2nd most common disease after leukemia.
More recently, scientists have argued that lymphogranulomatosis has an infectious etiology. Some researchers have suggested that the causative agent of this disease is Koch's bacillus, a bacterium that causes tuberculosis. Also, the cause of the development of the disease was called streptococcal, diphtheria infection, or the abnormal activity of pale spirochetes and E. coli. There were theories of viral origin of Hodgkin's disease, but they, too, were refuted.
To date, it has been possible to establish that leukemias and hematosarcomas occur against the background of a disturbance in the functioning of the blood formation system. And the specific cause of Hodgkin's disease is the presence of malignant Berezovsky-Sternberg cells.
Also, factors predisposing to this disease (for example, work in harmful enterprises, addiction, improper lifestyle, etc.) are not fully established. According to some studies, the risk of developing Hodgkin's disease is increased in patients who have undergone infectious mononucleosis or dermatological diseases. Also at risk include people working in sewing or woodworking enterprises in the agricultural sector. Doctors and chemists are also susceptible to this disease.
Cases of the development of Hodgkin's disease have been reported in several members of the same family. Based on this, it is possible to conclude that a weakly virulent infection of viral origin, as well as a person’s genetic susceptibility to this disease. However, this fact has not yet been fully confirmed, therefore it remains only one of the theories of the development of a dangerous disease.
Lymphogranulomatosis can occur in a local and generalized form. In the first case, only one specific group of lymph nodes is affected. In the generalized form of the disease, proliferative processes occur in the tissues of the spleen, liver, stomach, lungs, and even in the thick layers of the skin.
Localization of lymphogranulomatosis is:
For the duration of the development of Hodgkin's disease is:
- acute, which lasts for several months before the onset of the terminal phase;
- chronic, protracted, in which the pathological process in the human body has a sluggish, but persistent nature, and can occur for many years.
According to the morphological structure and proportion of pathological cells, lymphogranulomatosis is divided into:
- lymphohistiocytic (lymphoid dominance);
- nodular sclerotic - nodular sclerosis;
- mixed cell;
- lymphoid depletion.
Hodgkin's disease goes through 4 stages of development:
- At the first, local, stage, only one specific group of lymph nodes is affected, or 1 extra lymphatic organ (IE).
- The second stage is regional, in which the disease affects 2 or more groups of lymph nodes located on one side of the diaphragm (II). Also, one extra lymphatic organ and regional lymph nodes (IIE) may be involved in the pathological process.
- In the third, generalized stage, the lymph nodes on both sides of the diaphragm are affected. In parallel, the disease can affect 1 extra lymphatic organ, as well as the spleen. In some cases, both organs may be involved in the pathological process.
- The last, fourth, stage is called disseminated. At this time, 1 or more extra-lymphatic organs are affected. The pathological process can take place in the pleura, lung, bone marrow, liver, kidneys, gastrointestinal tract organs, etc. At the same time, the lymph nodes can be either affected or remain in a healthy state.
The diagnosis takes into account the patient’s disturbing symptoms for half a year. In this case, along with the stage of the disease, the letters A or B are put down.
Symptoms of Hodgkin's disease
Symptoms of Hodgkin's disease can manifest themselves in different ways. But most often patients express complaints about the presence of:
- enlarged lymph nodes in a specific part of the body;
The disease begins, as a rule, gradually, so its clinical manifestations may be blurred, or else be completely absent. First of all, there is a painless increase in lymph nodes, while they are not soldered to neighboring tissues or organs. If the LU in the mediastinum area increases in parallel, this can lead to the development of coughing, shortness of breath, as well as problems with swallowing.
Quite often, pathology begins with fever and hyperhidrosis. The patient complains of sudden changes in temperature, while it can rise sharply to 39 degrees, and then self-normalize. In parallel with this, the patient is tormented by bouts of sweating, which occur mainly at night.
All these symptoms are caused by intoxication of the body. It is also triggered by the decay products of pathological tumor cells. In 10-50% of cases, patients feel unreasonable bouts of itching on the body.
The typical development of Hodgkin's disease is characterized by a primary lesion of the mediastinum with permeable growth of lymph nodes. This affects the pleura, lungs, and also accumulates fluid in the pleural cavity. This is what leads to shortness of breath, cough, chest pain.
To begin with, a thorough history is conducted with a full study of the patient’s disturbing symptoms. After that, the functioning of the spleen, liver is evaluated. The patient then goes to a chest x-ray, CT scan and MRI.
In the blood analysis of patients with Hodgkin's disease, the presence of can be traced:
- iron deficiency anemia;
- increased ESR along with pancytopenia - in the later stages of the disease;
- monocytosis, neutrophilic leukocytosis, eosinophilia - also mainly in the late stages of the pathology.
Sometimes in the leucocentrate, Berezovsky-Sternberg cells can be detected.
An immunogram is performed along with a complete blood test. It notes the slowing down of immune responses, as well as the inhibition of B-lymphocyte synthesis. Such a deviation is noted in the late stages of Hodgkin's disease.
The results of urine tests remain normal. If kidney damage occurs, there may be protein and red blood cell impurities in the urine.
For biochemical analysis of blood in Hodgkin's disease, the following disorders are characteristic:
- Increased bilirubin and enzymes. This deviation is observed with liver damage.
- Perhaps an increase in seromukoidov, haptoglobin, globulins.
At WG an increase in intrathoracic and cervical LU can be recorded. This research method is very effective in the early diagnosis of Hodgkin's disease. These diagnostic procedures are considered the most informative in this disease. With the help of CT, lymphomas can be detected in any organ or part of the human body.
With the help of MRI, you can more accurately identify the localization and determine the size of the tumor. In addition, this study is safer compared to CT, so it can be carried out regularly to monitor the effectiveness of therapy.
The point in the diagnosis can be put using a tissue biopsy of an enlarged lymph node. Histological examination of the biopsy can be combined with a bone marrow biopsy. At an early stage of the disease, its structure will be unchanged. In the generalized form of Hodgkin's disease, a delay in the development of erythrocyte cells is detected, lymphogranulomatosis foci are detected, Berezovsky-Sternberg cells are detected, and a tendency to sclerosis is recorded. During the biopsy of the lymph nodes, specific cells are detected, as well as polymorphic cell granulomas.
Treatment of Hodgkin's disease
Today, treatment for Hodgkin's disease is carried out in 3 ways:
- radiation treatment;
- combination of the two previous methods.
In severe cases, chemotherapy is performed using high doses of drugs. After the end of the course of treatment, the patient undergoes a bone marrow transplant.
At stage 1-2 of the disease, in the absence of pronounced symptoms, radiation therapy is performed using radiation at a dose of 3600-4400 cGy (1000-cGy weekly). With this approach, in 85% of cases, it is possible to achieve stable remission of Hodgkin's disease.
Radiation therapy is carried out using special medical devices. Only affected LU groups are irradiated. At the same time, the negative impact of such treatment on healthy cells is neutralized with the help of special lead protective filters.
A large number of schemes have been developed for chemotherapy. The most commonly used is the BEASORR scheme, implying the use of:
- Doxorubicin (Adriamycin);
- Vincristine (Oncovin);
A variation of this chemotherapeutic complex is BEACOPP-esc. Such a treatment regimen implies an increase in doses of certain components in comparison with BEASORR. Another approach is WESORR-14. The principle of therapy is almost completely identical to BEASORR, however, in this case the interval between courses is only 2 weeks.
In Hodgkin's disease, more outdated chemotherapy regimens can be used in the form of the Stanford IV protocol, Stanford V protocol, COPP, ABVD schemes with Doxorubicin, Bleomycin, Vinblastine, Dacarbazine. One cycle is designed for 28 days, therapy continues, on average, six months.
The MORR scheme, in comparison with the CORP, has one drawback - the risk of developing leukemia in a patient who has undergone therapy increases. The onset of pathology is noted approximately 5-10 years after treatment.
Combination of radiotherapy and chemotherapy is considered to be the most effective in the fight against lymphogranulomatosis. With this therapeutic approach, it is possible to achieve remissions lasting from 10 to 20 years. Such progress is noted in 90% of cases.
Often, treatment of patients begins in the hospital, but if the patient is well tolerated by the therapy, it can be continued on an outpatient basis.
In 2016, a new drug, Adzetris, was registered in Russia. It is intended for the treatment of CD30-positive lymphomas:
- Patients who underwent 2 lines of chemotherapy in the treatment of Hodgkin's disease. Also, the drug can be used for patients with lymphogranulomatosis who have undergone autologous transplantation. In some cases, the drug is prescribed to people who have undergone non-transplant therapy and who are not candidates for medical reasons.
- In patients with systemic anaplastic large cell lymphoma who have undergone at least 1 line of chemotherapy.
Adsetris is a conjugate of an antibody and a cytotoxic agent. It is characterized by a selective effect on tumor cells.
For the treatment of recurrent lymphogranulomatosis, starting in 2017, a new immunological preparation Keitrud is used.
Lymphogranulomatosis is a neoplastic disease that can be completely cured. However, only under the condition of timely access to a doctor and undergoing a full course of therapy. Full recovery is noted in 80% of cases.
Prognosis for lymphogranulomatosis depends on:
- Stages of the pathological process. When a disease is detected at stages 1–2 of development and prescription of a complex radiation and chemotherapeutic treatment, recovery occurs in 90% of patients. When Hodgkin's disease is detected at stages 3–4, the prognosis becomes less favorable. It is possible to transfer the disease to the remission phase only in 80% of patients.
- Histological structure of the tumor. Lymphohistiocytic tumor and nodular sclerosis leads to a change in the structure of the LN, but their partial recovery is possible. Mixed cell lymphoma and lymphoid depletion lead to irreversible processes in the lymph nodes. In parallel, the number of lymphocytes in the body decreases, and this is a very unfavorable deviation.
- Target organ damage. Metastasis to other internal organs leads to disruption of their work. Quite often, this leads to irreversible consequences and serious complications.
- The presence and frequency of relapses. 10–30% of patients experience repeated outbreaks of the disease even after radical chemotherapy. Such relapses are considered unfavorable from a prognostic point of view.
- The effectiveness of therapy. In rare cases, Hodgkin's disease occurs in forms that are resistant to any type of treatment.
In lymphogranulomatosis, the patient must be registered with an oncologist and a hematologist.
Of great importance in the prevention of Hodgkin's disease is the annual passage of preventive medical examinations. Sometimes the primary foci of the disease can be detected using fluorography, so this procedure can not be neglected.
Hodgkin's disease is often found in children, so hardening plays an important role in its prevention, avoiding prolonged exposure of the child to UV radiation, preventing exposure of the child's body to radiation and harmful chemicals.
As for elderly patients, to prevent the development of Hodgkin's disease, it is necessary to abandon electrotherapy, as well as to limit the conduct of certain physiotherapeutic procedures.