Hodgkin's lymphoma
The content of the article:
- Causes of Hodgkin's lymphoma
- Forms of the disease
- Stages of Hodgkin's lymphoma
- Hodgkin's lymphoma symptoms
- Diagnostics
- Hodgkin's lymphoma treatment
- Potential consequences and complications
- Forecast
- Prevention
Hodgkin's lymphoma (lymphogranulomatosis, Hodgkin's disease) is a malignant tumor of lymphoid tissue with the formation of specific polymorphic cell granulomas. The tumor substrate of Hodgkin's lymphoma is Reed-Sternberg cells (lacunar histiocytes) - large polyploid cells containing a multilobular nucleus. The bulk of the tumor of the affected lymph node is a granuloma with accumulations of lymphocytes (T cells predominate among them), granulocytes, histiocytes, eosinophils, plasma and reticular cells. The tissue of the affected lymph node is permeated with fibrous connective tissue strands emanating from the capsule.
Source: okeydoc.ru
The disease is named after Thomas Hodgkin, who in 1832 described seven cases of the disease and proposed to separate pathology into a separate nosological unit, the common feature of which is enlargement of the lymph nodes and spleen, cachexia (extreme exhaustion of the body) and death.
The average incidence of Hodgkin's lymphoma ranges from 0.6–3.9% in men and 0.3–2.8% in women, and averages 2.2 cases per 100,000 population. The disease occurs at all ages; among childhood cancers, it ranks third in prevalence.
Causes of Hodgkin's lymphoma
The etiological factor of the disease remains unclear. It is assumed that Hodgkin's lymphoma is a B-cell tumor that develops against the background of T-cell immunodeficiency due to hypofunction of the thymus.
There are several predisposing factors:
- infectious diseases - cells of the lymphatic system begin uncontrolled division and undergo mutations under the influence of viruses (herpeviruses, retroviruses, etc.);
- congenital diseases of the immune system - Louis-Bar syndrome, Wiskott-Aldrich syndrome, etc.;
- autoimmune diseases - rheumatoid arthritis, Sjogren's syndrome, celiac disease, systemic lupus erythematosus, etc.;
- genetic predisposition - no genetic markers have been identified, but there is an increase in the frequency of Hodgkin's lymphoma in families where such diseases have already been diagnosed;
- the influence of carcinogenic chemicals - benzenes, aniline dyes, heavy metal salts, aromatic hydrocarbons, pesticides, etc.;
- work associated with exposure to increased radiation, high frequency currents, irradiation.
Forms of the disease
The classification of Hodgkin's lymphoma is based on the histological characteristics of the affected tissue.
There are four histological variants of Hodgkin's lymphoma:
- nodular (nodular) sclerosis (types 1 and 2) is the most common form of the disease, accompanied by the formation of collagen strands in the lymph nodes inside the chest cavity, which divide the formed tumor tissue into many rounded sections. Reed-Sternberg cells are revealed;
- lymphohistiocytic (lymphoid predominance) - a classic variant of Hodgkin's lymphoma, which is characterized by the presence of a large number of lymphocytes in the affected organ. Reed - Sternberg cells are single, Hodgkin cells are often found. Clusters of lymphocytes merge with each other and form areas of diffuse growth, fibrosis and necrosis are absent. The most often affected are the cervical axillary, inguinal lymph nodes;
- lymphoid depletion - Reed-Sternberg cells predominate in the affected tissue, between them single disseminations of lymphocytes are noticeable, the level of which is constantly decreasing. The variant of lymphoid depletion usually corresponds to the IV stage of the spread of the disease and is characterized by an unfavorable course;
- mixed cell variant - the histological picture is represented by a large number of lymphocytes, eosinophils, plasmocytes, Reed-Sternberg cells in the affected tissue. Foci of necrosis, fields of fibrosis are often found.
Source: hematology.org
Stages of Hodgkin's lymphoma
When establishing the stages of Hodgkin's lymphoma, the data of examination and biopsy results are taken into account, the number of organs and tissues involved in the pathological process is determined, the prevalence of the process is above or below the diaphragm:
- I - one group of lymph nodes is affected;
- IE - I + involvement of one extra-lymphatic organ in the pathological process;
- II - two or more groups of lymph nodes are affected on one side (above or below) of the diaphragm;
- II E - lesion of the lymphoid organ with an increase in 1-2 groups of lymph nodes, localization of the lesion - on the same side relative to the diaphragm;
- III - several groups of lymph nodes are affected on both sides (top and bottom) of the diaphragm;
- III S - lesion of the spleen joins;
- III E - III + localized lesion of an extra-lymphatic organ or tissue;
- IV - diffuse or disseminated (multifocal) lesion of one or more internal organs, which may be accompanied by damage to the lymph nodes.
Stage IV can have the following course options:
- A - there is no intoxication;
- B - signs of intoxication, weight loss over the past six months;
- a - there is no biological activity in terms of blood tests;
- b - biological activity is detected.
Hodgkin's lymphoma symptoms
Initially, the pathological process develops in the lymph nodes. They increase gradually, the tumor process spreads and affects other organs and tissues. In the early stages, the disease is usually asymptomatic. As the lymph nodes grow, they become sore, and symptoms appear associated with compression of the surrounding tissues and organs.
Local signs of Hodgkin's lymphoma:
- enlarged lymph nodes;
- damage to internal organs.
The main symptom of Hodgkin's lymphoma is lymphadenopathy (characterized by a significant increase in lymph nodes). The lymph nodes of the middle and anterior mediastinum, sometimes the thymus, are affected. Also, the lesion can affect any organs, the spleen, liver, skin, bone marrow, lungs, pleura, bone tissue can be involved in the process.
Common symptoms of Hodgkin's lymphoma:
- fever with an undulating character;
- increased sweating at night (profuse night sweats);
- weakness, apathy;
- lack of appetite;
- itchy skin;
- sudden weight loss for no reason, weight loss can reach critical levels;
- anemia;
- decreased immunity, susceptibility to infectious diseases.
With a pronounced increase in the lymph nodes of the chest, the following symptoms develop:
- dry, unproductive cough;
- hoarseness of the voice;
- a feeling of heaviness in the affected area;
- dysphagia (swallowing disorder);
- dyspnea (shortness of breath);
- kava syndrome (circulatory disorders in the basin of the superior vena cava);
- pleurisy, pericarditis.
When the process becomes widespread, signs of damage to internal organs appear. Most often, damage to the lymph nodes of the spleen and liver is found. As a result of the increase in these internal organs, the stomach is compressed and the kidneys are displaced. The involvement of the lymph nodes is manifested by the appearance of abdominal pain of varying intensity.
The defeat of the lungs, as a rule, is of a secondary nature and is a consequence of the transition of the process from the lymph glands of the mediastinum to the lungs. The accumulation of fluid in the pleural cavities is often detected.
Damage to the nervous system is detected after extensive spread of lymphogranulomatosis in the lymph glands and internal organs. The most common lesions of the spinal cord, in which lymphogranulomatous elements grow in the epidural tissue and compress the spinal cord. In such cases, the disease proceeds as a tumor of the spinal cord with conduction disorders of sensitivity, paresis and paralysis, pain in the extremities.
The two main ways of damage to bone tissue are lymphohematogenous spread and invasion of lymphogranuloma into bone tissue. Primary bone lesions are detected during the first year of the disease, secondary changes in the bones appear after 1.5–2.5 years from the onset of the disease. Skeletal changes occur when the process spreads from the adjacent lymph nodes, pleura, mediastinum. Lymphogranulomatous tissue causes destruction of bone structure and osteosclerosis. The pressure of the enlarged lymph nodes on the adjacent nerve plexuses is manifested by intense pain in the spine, in the affected bones of the skeleton.
Signs of the biological activity of the process due to the production of cytokines are also noted: an increase in the level of serum haptoglobin, the erythrocyte sedimentation rate, the content of ceruloplasmin and lactate dehydrogenase, the concentration of fibrinogen above the reference values. The appearance of signs of biological activity during the period of remission indicates an incipient exacerbation.
Diagnostics
Early detection of the disease is difficult due to the fact that clinical symptoms do not have a strictly defined character, and are often completely absent.
Diagnosis of Hodgkin's lymphoma is based on the morphological picture of the affected lymph node or organ. A number of studies are assigned:
- clinical examination and collection of anamnesis - special attention is paid to the symptoms of intoxication, palpation of all peripheral groups of lymph nodes, spleen and liver, examination of the nasopharynx, tonsils is carried out;
- biopsy of the affected lymph nodes with histological and immunohistochemical analysis of the biopsy. The criterion for Hodgkin's lymphoma is the presence in the material taken of specific Reed-Sternberg cells, immature Hodgkin cells;
- laboratory tests - general and biochemical blood test, erythrocyte sedimentation rate, serum alkaline phosphatase activity, renal and liver function tests. In the clinical analysis of blood, an increase in ESR, neutrophilic leukocytosis is found, moderate eosinophilia, an increase in the concentration of fibrinogen, thrombocytosis, and a decrease in the content of albumin are possible. At the initial stage of the disease, blood tests reveal moderate leukocytosis, which, as the disease progresses, is replaced by leukopenia;
- laboratory assessment of thyroid function - with damage to the cervical lymph nodes;
- immunophenotypic studies of tumor tissue - identify qualitative and quantitative violations of the T-cell link of immunity.
- X-ray examinations of the chest, skeleton, gastrointestinal tract - are of leading importance in determining the nature and localization of lesions;
- Abdominal ultrasound - for the same purpose as radiography;
- computed tomography, magnetic resonance imaging of the neck, chest organs, abdomen and pelvis - allows you to identify the presence of tumor formations in different parts of the body;
- trepanobiopsy - performed if there is a suspicion of damage to the bone marrow of the iliac bones;
- osteoscintigraphy - with an increased level of alkaline phosphatase in the blood serum;
- diagnostic laparotomy - used to take biopsies of mesenteric and para-aortic lymph nodes.
Hodgkin's lymphoma treatment
Various schemes of therapy have been developed, their choice is carried out taking into account the type and degree of damage, the duration and severity of the disease, the presence of concomitant pathology.
The general treatment regimen for lymphoma includes two stages:
- stimulation of remission using cyclic chemotherapy;
- consolidation of remission through radical radiation therapy and maintenance cycles of drug therapy.
When planning the volume of treatment, the unfavorable factors that determine the severity and prevalence of the tumor process are taken into account:
- involvement of three or more zones of lymphatic collectors;
- massive damage to the spleen and / or mediastinum;
- the presence of an isolated extranodal lesion;
- an increase in ESR of more than 30 mm / h in the presence of symptoms of intoxication and more than 50 mm / h in their absence.
Various options for radiation therapy are used - from local irradiation of primary lesions in reduced doses to irradiation according to a radical program of all lymph nodes at stage IV A. To prevent the spread of tumor cells through the lymphatic system, irradiation of the regional parts adjacent to the lesions is performed.
Polychemotherapy consists in the simultaneous use of several cytostatics. There are different combinations of chemotherapy (protocols), drugs are prescribed in long courses. Treatment is phased, provides for two-week cycles at two-week intervals, after the completion of six cycles, supportive treatment is prescribed.
Preference is given to chemotherapy in combination with radiation, which gives the maximum effect with the minimum number of complications. First, introductory polychemotherapy is performed with irradiation of only enlarged lymph nodes, then - irradiation of all other lymph nodes. After radiation exposure, maintenance chemotherapy is carried out according to one scheme or another. Intensive treatment during the period of improvement reduces the number of late complications and increases the possibility of treating exacerbations.
If the process is local and available for surgical intervention, the spleen, isolated lymph nodes, and single conglomerates of lymph nodes are removed, followed by irradiation on gamma-therapeutic devices. Splenectomy is also indicated for severe hematopoiesis depression, which prevents cytostatic treatment.
In case of progression of the disease and ineffectiveness of therapy, bone marrow transplantation is indicated.
Potential consequences and complications
Hodgkin's disease can have the following complications:
- sepsis;
- brain or spinal cord cancer;
- bleeding;
- pressure of the neoplasm on the airways, leading to asphyxiation;
- superior vena cava syndrome;
- the development of obstructive jaundice (with compression of the bile duct);
- immunological shifts;
- intestinal obstruction (when the intestines are compressed by lymph nodes);
- cachexia;
- dysfunction of the thyroid gland;
- fistula formation of peripheral lymph nodes;
- myocarditis and pericarditis;
- secondary malignant neoplasms;
- violation of protein metabolism of the kidneys and intestines;
- side effects of chemotherapy and radiation.
Forecast
Adequate and timely treatment allows to obtain good results; more than 50% of patients manage to achieve stable remission. The effectiveness of therapy is predetermined by a differentiated approach to the development of treatment regimens for different groups of patients, identified on the basis of unfavorable prognostic factors.
With intensive programs, the clinical effect is usually observed already during the first cycle. In the treatment of patients with early and generalized stages of Hodgkin's lymphoma, the 5-year progression-free survival rate is 90%, with stage III - 60–80%, at stage IV remission reaches less than 45% of patients.
Unfavorable prognostic signs:
- massive conglomerates of lymph nodes more than 5 cm in diameter;
- lymphoid depletion;
- simultaneous damage to three or more groups of lymph nodes;
- expansion of the mediastinal shadow by more than 30% of the chest volume.
Prevention
Special measures for the primary prevention of Hodgkin's disease have not been developed. Special attention is paid to secondary prevention - relapse prevention. People who have had Hodgkin's lymphoma are advised to exclude physical, electrical and thermal procedures, avoid physical overload, insolation, they are contraindicated in work associated with exposure to harmful industrial factors. To prevent a decrease in the number of leukocytes, blood transfusions are performed. In order to prevent adverse events, women are advised to plan a pregnancy no earlier than two years after recovery.
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Anna Kozlova Medical journalist About the author
Education: Rostov State Medical University, specialty "General Medicine".
The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!
