Nodular sclerosis is a histological type of lymphogranulomatosis, characterized by dense growth of connective tissue, divided by a mass of irregularly shaped cells and lobules. They contain overgrown lymphoid matter with a huge number of Berezovsky-Sternberg cells. The disease begins with an increase in nodes. This pathology is one of the variants of classical Hodgkin lymphoma.
Hodgkin's disease is considered a serious ailment affecting the lymphatic system. The disease can form in any organ that has lymphoid tissue (thymus, tonsils, spleen, adenoids, etc.).
Nodular sclerosis: symptoms
Hodgkin's lymphoma can be in a person if he has such manifestations as:
- weight loss;
- swollen lymph nodes (often in the neck);
- loss of appetite;
- dyspnea;
- night sweats or temperature;
- chest pain
- enlarged liver (5% of patients) or spleen (30% of patients);
- severity or pain in the abdomen (in children);
- itchy skin (only in 1/3 of people suffering from an ailment);
- difficulty breathing
- cough.
Causes
Lymphogranulomatosis can be infected at any age, but more often it is observed in young men of the age category from 16 to 30 years old or in older people over 50 years old. Children under 5 years old practically do not get sick. What specifically provokes this disease is still unknown. However, there is an assumption that the source is viruses. It is believed that the onset of this ailment can:
- immunodeficiency states;
- infectious mononucleosis (excited by the Epstein-Barr virus).
Nodular sclerosis of Hodgkin's lymphoma can pass instantly, last from 3 to 6 months or stretch for 20 years.
What stages does the disease have?
The degrees of Hodgkin's lymphoma are determined by laboratory tests and based on the following indicators:
- the number of affected lymph nodes, as well as their location;
- the presence of these nodes in different areas of the diaphragm;
- tumors in other organs (for example, in the liver or spleen).
First stage. In this case, only one lymph node or lymphoid organ (spleen, Pirogov-Walder ring) is affected.
Second stage. Here, lymph nodes on both sides of the chest, diaphragm, and lymphoid organs are usually affected.
Third stage. This degree of Hodgkin lymphoma is almost no different from the second stage. However, it has two types of nodular sclerosis of the third stage:
- in the first case, organs located below the diaphragm are affected (celiac lymph nodes, spleen);
- in addition to the areas that are listed in the first variety, other places with lymph nodes located near the diaphragm are also affected.
Fourth stage. Not only nodes are affected, but also non-lymphoid organs - bone marrow, liver, bones, lungs and skin.
Hodgkin lymphoma degree designations
The indicator of the severity of the clinical situation and the painful course of other tissues and organs is marked with letters.
A - there are no severe general manifestations of the disease.
B - the presence of one or more symptoms (causeless increased temperature, sweating at night, rapid weight loss).
E - foci spread to tissues and organs located near the affected lymph nodes.
S - there is a lesion of the spleen.
X - there is a serious tumor of huge sizes.
Histological types of disease
Regarding the cell structure of lymphogranulomatosis, 4 forms of malaise are distinguished.
- Nodular sclerosis of Hodgkin's lymphoma is the most common form of the disease, approximately 40-50% of all cases. Most often, young women are affected by them, in which the lymph nodes of the mediastinum are mainly affected. In the biopsy material, in addition to Berezovsky-Sternberg cells, there are also large lacunar cells with foamy cytoplasm and a mass of nuclei. The prognosis for this disease is usually good.
- Lymphohistiocytic lymphoma, which forms in 15% of the examples. More often it can be found in young men under 35 years old. It has an excellent five-year survival and has cells of mature lymphites, as well as Shtrenberg. This type of ailment is of small malignancy and it is detected in the initial stages.
- The combined species is usually diagnosed in the elderly and children. It is characterized by a characteristic typical clinical picture and a tendency to generalize action. A histological examination reveals different variants of cell connections, including Sternberg. Found in 30% of patients with lymphoma. Nodular sclerosis in this case has a relatively good prognosis, and if treatment is prescribed on time, a solid remission occurs.
- Dangerous granuloma with the destruction of lymphoid tissue is noted infrequently, only in 5% of examples (mostly among the elderly). A characteristic feature here is that there are no lymphocytes and Sternberg cells predominate. This form of lymphoma is characterized by the lowest percentage of five-year survival.
Diagnostics
The diagnosis of lymphoma is determined only by histological examination of the lymph nodes and is considered proven only if, as a result of this study, special multinuclear Sternberg cells were found. In severe examples, immunophenotyping is needed. Cytological analysis of the lymph node or kidney puncture is usually small in order to confirm type 1 nodular sclerosis. What needs to be done to establish a diagnosis of the disease:
- general and biochemical blood analysis;
- X-ray of the lungs (without fail in lateral and direct projection);
- lymph node biopsy;
- ultrasound examination of all types of peripheral and intra-abdominal lymph nodes, thyroid gland, liver and spleen;
- computed tomography of the mediastinum to eliminate invisible lymph nodes in traditional radiography;
- trepan biopsy of the ilium to exclude bone marrow damage;
- scanning and radiography of bones.
Therapy
Contains radiation treatment, surgical interventions and chemotherapy. The choice of method is established by the stage of malaise and the presence of positive or negative prognostic causes. Favorable factors include:
- nodular sclerosis and lymphohistiocytic type detected by histological examination;
- age less than 40 years;
- volumes of lymph nodes that do not exceed 6 cm in diameter;
- lack of common manifestations of biological effectiveness (development of biochemical blood parameters);
- the presence of no more than 3 lesion locations.
If at least one of these causes is absent, then the patient is assigned to the group with an unfavorable prognosis.
Radiation treatment
Complete radiation therapy as an individual method is used for patients with IA and IIA stages, confirmed by laparotomy, and having good prognostic factors. It is made free fields with irradiation of any types of affected lymph nodes, as well as passages of the outflow of lymph.
The total absorbed portion in the metastases of the lesion is 40-45 g in 4-6 weeks, in places of prophylactic exposure - 30-40 g in 1-4 weeks. Also, with wide-field methods of multi-field irradiation of some foci are used to prevent nodular sclerosis ns1.
Radiation treatment can cause complications such as fibrosis of the subcutaneous tissue, radiation pulmonitis and pericarditis. Deterioration appears in a different period - from 3 months to 5 years after therapy. Their complexity depends on the dose used.
Operations
Surgical treatment is rarely used alone, it is usually an integral part of therapy in the complex. Perform splenectomy, as well as surgery on the trachea, esophagus, stomach and other organs (with the risk of asphyxiation, eating disorders). Pregnancy detected with ongoing lymphogranulomatosis must be terminated.
Chemotherapy
This type is used as one of the components of complex treatment. To cure nodular sclerosis, different drugs are used:
- alkaloids ("Vinblastine" or "Rosevine", "Etoposide" or "Vincristine," Oncovin ");
- alkylating mixtures (Mustargen, Cyclophosphamide or Embihin, Nitrosomethylurea or Chlorobutin);
- synthetic agents ("Natulan" or "Procarbazine", "Dacarbazine" or "Imidazole-Carboxamide");
- antitumor antibiotics ("Bleomycin", "Adriablastin").
Monochemotherapy
It is used only in special cases with indicative purposes. As a rule, several medications are prescribed with a different mechanism of action (polychemotherapy). In the fourth stage, in patients with diffuse lesions of the liver or bone marrow, this type of treatment is the only way - this is classical Hodgkin's lymphoma. Nodular sclerosis is treated according to such schemes:
- ABVD (Bleomycin, Dacarbazine, Adriablastin, Vinblastine);
- MOPP ("Onkovin", "Prednisolone", "Mustargen", "Procarbazine");
- CVPP (Vinblastine, Prednisone, Cyclophosphamide, Procarbazine).
Therapy is carried out by short-term (2, 7, 14 days) courses with two-week breaks. The number of cycles varies due to the magnitude of the initial lesion and the susceptibility to treatment. Usually a complete remission is achieved with the prescription of 2-6 courses. After this, it is recommended to perform another 2 cycles of therapy. If the result was a partial remission, then the treatment regimen changes, and the number of courses increases.
Taking medications is accompanied by hematopoiesis pressure, alopecia, dyspeptic manifestations that go away at the end of treatment. Nodular sclerosis also leads to such late complications as infertility, leukemia and other malignant tumors (secondary tumors).
Forecast
It is caused by the features of the course of lymphogranulomatosis, the clinical stage of the disease, the patient's age, histological appearance and others. With a sharp and subacute disease process, the prognosis is not good: patients usually die in the period from 1-3 months to 1 year. But with chronic lymphogranulomatosis, the prognosis is conditionally positive. The disease can last a very long time, up to 15 years (in some cases, much longer).
In 40% of all infected, especially at the 1st and 2nd stages, as well as favorable prognostic reasons, for 10 years or more relapses were not observed. Disability as a result of prolonged remissions is not impaired.
Prevention
As a rule, it is aimed at preventing relapse. Patients with lymphogranulomatosis are subject to an oncologist examination. In the study, which is required to be performed every six months for the first 3 years, and then once a year, it is necessary to focus on biological indicators of effectiveness, which are often the initial signs of relapse (an increase in the level of fibrinogen and globulins, an increase in POPs). Thermophysiological procedures, overheating and direct insolation are harmful to patients with lymphogranulomatosis. An increase in the number of relapses due to pregnancy has been established.
Now many people already know for sure that Hodgkin’s lymphoma is a variant of nodular sclerosis, which is a very unpleasant and intractable ailment.