Nodular sclerosis is a histological variety of lymphogranulomatosis, characterized by a dense growth of connective tissue, dividing into 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's 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 gland, tonsils, spleen, adenoids, etc.).
Nodular sclerosis: symptoms
Hodgkin's lymphoma can be in a person if he has symptoms such as:
- weight loss;
- swollen lymph nodes (often in the neck area);
- loss of appetite;
- shortness of breath;
- night sweats or fever;
- chest pain;
- enlarged liver (5% of patients) or spleen (30% of patients);
- heaviness or pain in the abdomen (in children);
- skin itching (only 1/3sick people);
- hard breathing;
- cough.
Reasons
Lymphogranulomatosis can be contracted at any age, but it is more common in young men between the ages of 16 and 30 or in older people over 50. Children under 5 years of age practically do not get sick. What exactly 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 be:
- immunodeficiency states;
- infectious mononucleosis (caused by the Epstein-Barr virus).
Nodular sclerosis of Hodgkin's lymphoma can resolve instantly, last from 3 to 6 months, or stretch for 20 years.
What are the stages of the disease?
Hodgkin's lymphoma grades are determined by laboratory results and based on the following indicators:
- number of affected lymph nodes and their location;
- the presence of these nodes in different areas of the diaphragm;
- tumors in other organs (for example, in the liver or spleen).
The first stage. In this case, only one lymph node or lymphoid organ is affected (spleen, Pirogov-Walder ring).
Second stage. Lymph nodes on both sides of the chest, diaphragm and lymphoid organs are usually affected here.
Third stage. This degree of Hodgkin's lymphoma is almost the same as the second stage. However, she has two types of nodular sclerosisthird stage:
- in the first case, organs located below the diaphragm (abdominal lymph nodes, spleen) are affected;
- in addition to the areas listed in the first variety, other places with lymph nodes located near the diaphragm are also affected.
The fourth stage. Not only nodes are affected, but also non-lymphoid organs - bone marrow, liver, bones, lungs and skin.
Designations of degrees of Hodgkin's lymphoma
The indicator of the severity of the clinical situation and the painful course of other tissues and organs is marked with letters.
A - no severe general manifestations of the disease.
B - one or more symptoms present (unexplained fever, night sweats, rapid weight loss).
E - lesions 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 size.
Histological types of disease
Regarding the cellular structure of lymphogranulomatosis, there are 4 forms of malaise.
- Nodular sclerosis of Hodgkin's lymphoma is the most common form of the disease, accounting for approximately 40-50% of all cases. They are most often affected by young women, who are mainly affected by the lymph nodes of the mediastinum. In the biopsy material, in addition to Berezovsky-Sternberg cells, there are also large lacunar cells with foamy cytoplasm and a mass of nuclei. Forecasting with thissickness is usually good.
- Lymphohistiocytic lymphoma, which forms in 15% of cases. More often it can be found in young men under 35 years of age. It has an excellent five-year survival rate and has mature lymphaitis cells, as well as Strenberg cells. This type of disease with a small malignancy and it is detected in the initial stages.
- The combined variety is usually diagnosed in the elderly and children. It is distinguished by a characteristic typical clinical picture and a tendency to generalize the action. Histological examination reveals different variants of cell connections, including Sternberg. It is 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 without problems.
- Dangerous granuloma with destruction of lymphoid tissue is observed infrequently, only in 5% of cases (mostly among the elderly). A characteristic feature here is that there are no lymphocytes and Sternberg cells predominate. This form of lymphoma has the lowest five-year survival rate.
Diagnosis
The diagnosis of "lymphoma" is determined only by a histological examination of the lymph nodes and is considered proven only if, as a result of this study, special multinucleated Sternberg cells were found. In severe cases, immunophenotyping is needed. Cytological analysis of the lymph node or puncture of the kidneys is usually not enough to confirmnodular sclerosis type 1. What needs to be done to establish a diagnosis of the disease:
- general and biochemical blood tests;
- radiography of the lungs (mandatory in the lateral and direct projection);
- lymph node biopsy;
- ultrasound examination of all types of peripheral and intra-abdominal lymph nodes, thyroid gland, liver and spleen;
- mediastinal computed tomography to eliminate invisible lymph nodes on conventional radiography;
- trepan-biopsy of the ilium to rule out bone marrow damage;
- Scans and radiographs of bones.
Therapy
Contains radiation treatment, surgery and chemotherapy. The choice of method is determined by the stage of malaise and the presence of positive or negative prognostic factors. Favorable factors include:
- nodular sclerosis and lymphohistiocytic type detected by histological examination;
- under 40;
- volumes of lymph nodes that do not exceed 6 cm in diameter;
- absence of general manifestations of biological efficacy (development of biochemical parameters of blood);
- no more than 3 hit locations.
If at least one of these reasons is missing, then the patient is classified as having a poor prognosis.
Radiotherapy
Total radiotherapy as an individual method is used for patients withIA and IIA stages, confirmed at laparotomy, and having good prognostic factors. It is made free fields with irradiation of any kind of affected lymph nodes, as well as passages of lymph outflow.
The total absorbed portion in the metastases of the lesion is 40-45 g in 4-6 weeks, in the places of prophylactic irradiation - 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 subcutaneous fibrosis, radiation pulmonitis and pericarditis. Deteriorations appear in a different period - from 3 months to 5 years after therapy. Their complexity depends on the dose consumed.
Operations
Surgical treatment is rarely used separately, it is usually an integral part of therapy in the complex. Splenectomy is performed, as well as operations on the trachea, esophagus, stomach and other organs (if there is a risk of asphyxia, a disorder in the passage of food). A pregnancy detected with ongoing Hodgkin's disease 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 "Rozevin", "Etoposide" or "Vincristine," Onkovin");
- alkaline mixtures ("Mustargen", "Cyclophosphan" or "Embikhin", "Nitrosomethylurea" or "Chlorbutin");
- synthetic products ("Natulan" or"Procarbazine", "Dacarbazine" or "Imidazole-Carboxamide");
- Antineoplastic antibiotics (Bleomycin, Adriablastin).
Monochemotherapy
Used only in special cases with an indicative appointment. As a rule, therapy with several drugs with different mechanisms of action (polychemotherapy) is prescribed. At the fourth stage in patients with diffuse lesions of the liver or bone marrow, this type of treatment is the only way - this is classic Hodgkin's lymphoma. Nodular sclerosis is treated according to the following schemes:
- ABVD ("Bleomycin", "Dacarbazine", "Adriablastin", "Vinblastine");
- MOPP (Onkovin, Prednisolone, Mustargen, Procarbazine);
- CVPP (Vinblastine, Prednisolone, 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 size of the initial lesion and susceptibility to treatment. Usually a complete remission is achieved with the prescription of 2-6 courses. After that, it is recommended to perform 2 more cycles of therapy. If the result was a partial remission, then the treatment regimen is changed, and the number of courses is increased.
Medication is accompanied by hematopoietic pressure, alopecia, dyspeptic manifestations that disappear at the end of treatment. Nodular sclerosis also leads to such late complications as infertility, leukemia and other malignant tumors (secondary tumors).
Forecast
Determinedfeatures of the course of lymphogranulomatosis, the clinical stage of the disease, the patient's age, histological appearance, and others. With a sharp and subacute process of the disease, 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 for a very long time, up to 15 years (in some cases much longer).
In 40% of all infected, especially in the 1st and 2nd stages, as well as favorable prognostic reasons, for 10 years or more, relapses are not observed. The ability to work as a result of prolonged remissions is not disturbed.
Prevention
Usually aimed at relapse prevention. Patients with lymphogranulomatosis are subject to a dispensary examination by an oncologist. In the study, which for the first 3 years is required to be performed every six months, 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). Patients with lymphogranulomatosis are harmful to thermal physiotherapy, overheating and direct insolation. An increase in the number of relapses due to pregnancy has been established.
Now, for sure, many people know that Hodgkin's lymphoma is a variant of nodular sclerosis, which is a very unpleasant and intractable disease.
