Classical Hodgkin's lymphoma: nodular sclerosis

Nodular sclerosis is a histological variant of lymphogranulomatosis, characterized by a dense growth of connective tissue, divided into a mass of irregularly shaped cells and lobules. They contain an overgrown lymphoid matter with a huge number of Berezovsky-Sternberg cells. The disease begins with an increase in the nodes. This pathology is one of the variants of classical Hodgkin's lymphoma.

Hodgkin's disease is considered a serious ailment that affects the lymphatic system. The disease can be formed in any organ that has lymphoid tissue( thymus gland, tonsils, spleen, adenoids, etc.).

Nodular sclerosis: symptoms of

Hodgkin's lymphoma can be in a person if it has such manifestations as:

• weight loss;
• enlarged lymph nodes( often in the neck area);
• loss of appetite;
• shortness of breath;
• night sweating or fever;
• chest pain;
• enlarged liver( 5% of patients) or spleen( 30% of patients);
• severity or pain in the abdomen( in children);
• skin itching( only in 1/3 of people suffering from an ailment);
• difficult breathing;
• cough.

Causes of

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 the elderly over 50 years. Children under 5 years 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 malaise can:

• immunodeficiency states;
• infectious mononucleosis( is caused by the Epstein-Barr virus).

Nodular sclerosis of Hodgkin's lymphoma can take place instantly, last from 3 to 6 months or stretch for 20 years.

What stages does the disease have?

The degrees of Khodjin lymphoma are determined by the results of laboratory tests and on the basis of the following indices:

• of the number of affected lymph nodes, and their location;
• availability 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-Valder ring).

The second stage. Here, the lymph nodes on both sides of the chest, the diaphragm and the lymphoid organs are usually affected.

Third stage. This degree of Hodgkin's lymphoma almost does not differ from the second stage. However, it has two types of nodular sclerosis of the third stage:

• in the first case affects organs located below the diaphragm( celiac lymph nodes, spleen);
• in addition to the areas listed in the first species, other sites with lymph nodes near the diaphragm are also affected.

Fourth stage. Affected not only nodes, 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 flow of other tissues and organs is marked with letters.

A - severe common manifestations of the disease there.

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 dimensions.

Histological types of the disease

With regard to the cellular structure of lymphogranulomatosis, four forms of malaise are distinguished.

1. Nodular sclerosis of Hodgkin's lymphoma is the most common form of the disease, approximately 40-50% of all cases. They are often sick of young women, who are mostly 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 a foamy cytoplasm and a mass of nuclei. The prognosis for this disease is usually good.
2. Lymphogystiocytic lymphoma, which is formed in 15% of the examples. More often it can be found in young men under 35 years old. Has an excellent five-year survival rate and has cells of mature lymphites, as well as Strenberg. This type of ailment with a slight malignancy and it is revealed in the initial stages.
3. A combined variety is usually diagnosed in the elderly and children. It has a typical typical clinical picture and a tendency to generalize the action. At a histological examination, different variants of cell connections are identified, including Sternberg. It is found in 30% of lymphoma patients. Nodular sclerosis in this case has a relatively good prognosis, and if treatment is prescribed on time, there is no problem with a solid remission.
4. Dangerous granuloma with the destruction of lymphoid tissue is noted infrequently, in only 5% of the 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.

Diagnosis of

The diagnosis of "lymphoma" is determined only by histological examination of the lymph nodes and is considered proven only when as a result of this investigation, special multinuclear Sternberg cells were found. In severe examples, immunophenotyping is necessary. Cytological analysis of the lymph node or renal puncture is usually small in order to confirm type 1 nodular sclerosis. What needs to be done to establish the diagnosis of the disease:

• general and biochemical blood test;
• lung radiography( mandatory in lateral and direct projection);
• lymph node biopsy;
• ultrasound examination of all kinds of peripheral and intra-abdominal lymph nodes, thyroid, liver and spleen;
• computed tomography of 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, surgery and chemotherapy. The choice of the method is established by the stage of malaise and the presence of positive or negative prognostic reasons. To favorable factors belong:

• revealed on histological examination nodular sclerosis and lymphohistiocytic type;
• age is less than 40 years;
• volumes of lymph nodes that do not exceed 6 cm in diameter;
• lack of common manifestations of biological efficacy( development of biochemical indicators of blood);
• the presence of no more than 3 lesion locations.

If at least one of these reasons is missing, then the patient is considered to be in a group with an unfavorable prognosis.

Radiation treatment

Complete radiotherapy as an individual method is used for patients with IA and IIA stages, confirmed with laparotomy, and having good prognostic factors. It is made free fields with irradiation of any kinds of affected lymph nodes, as well as passageways of lymph drainage.

Total absorbed dose in lesion metastases is 40-45 g for 4-6 weeks, in places of preventive exposure - 30-40 g for 1-4 weeks. Also with the wide-field method, the methods of multipole irradiation of some foci are used to prevent nodular sclerosis ns1.

Radiation treatment can cause complications such as fibrosis of 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

Operative treatment is rarely used separately, it is usually an integral part of therapy in the complex. Perform splenectomy, as well as operations on the trachea, esophagus, stomach and other organs( with the danger of asphyxia, eating disorders).The pregnancy detected during the course of the lymphogranulomatosis must necessarily be interrupted.

Chemotherapy

This kind is used as one of the components of complex treatment. To cure nodular sclerosis, different drugs are used:

• alkaloids( "Vinblastine" or "Rozevin", "Etoposide" or "Vinkristin," Onkovin ");
• alkylating mixtures( Mustargen, Cyclophosphane or Embihin, Nitrosomethylurea or Chlorbutin);
• synthetic agents( Natulan or Procarbazine, Dakarbazine or Imidazole-Carboxamid);
• antitumor antibiotics( "Bleomycin", "Adriablastin").

Monochemotherapy

It is used only in special cases with an indicative purpose. As a rule, prescribe therapy with several medications with different mechanisms of action( polychemotherapy).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 classical Hodgkin's lymphoma. Nodular sclerosis is treated according to the following schemes:

• ABDV( "Bleomycin", "Dakarbazin", "Adriablastin", "Vinblastine");
• MOTAP( "Onkovin", "Prednisolon", "Mustargen", "Procarbazin");
• CIPP( Vinblastine, Prednisolone, Cyclophosphane, Procarbazine).

Therapy is carried out with short-term( 2, 7, 14 days) courses with two-week breaks. The number of cycles varies with the magnitude of the initial lesion and the susceptibility to treatment. Usually the remission is achieved with the prescription of 2-6 courses. After this, it is recommended to perform 2 more cycles of therapy. If the result was partial remission, then the treatment scheme changes, and the number of courses increases.

Taking medication is accompanied by blood pressure, alopecia, dyspeptic manifestations that go away at the end of treatment. Nodular sclerosis still leads to such late complications as infertility, leukemia and other malignancies( secondary tumors).

Forecast

It is determined by the peculiarities of the course of lymphogranulomatosis, the clinical stage of the disease, the age of the patient, the histological type and others. With a sharp and subacute process of the disease, the prognosis is not good: patients usually die between 1-3 months and up to 1 year. But with chronic lymphogranulomatosis, the prognosis is conditionally positive. The disease can last very long, up to 15 years( in some cases much longer).

In 40% of all infected, especially in the 1 st and 2 nd stages, as well as favorable prognostic reasons, for 10 years and more relapses are not observed. The ability to work as a result of prolonged remissions is not violated.

Prevention

As a rule, it is directed to the prevention of relapses. Patients with lymphogranulomatosis are subject to on-site examination of the oncologist. In a study that requires the first 3 years to be performed every six months, and then once a year, it is necessary to focus on the biological performance indicators, which are often the initial signs of relapse( increased fibrinogen and globulin levels, increased POPs).Patients with lymphogranulomatosis are harmful thermal physiotherapy procedures, overheating and direct insolation. An increase in the number of relapses due to pregnancy has been established.

Now, for certain, many know that Hodgkin's lymphoma is a variant of nodular sclerosis, which is a very unpleasant and intractable ailment.