What is lymphogranulomatosis?

Lymphogranulomatosis is a serious oncological disease, characterized by pathological proliferation of lymphoid tissue and the appearance in it of specific cells of Berezovsky-Sternberg (structural elements of granulomas). This article will tell you about lymphogranulomatosis, symptoms, treatment, the causes of this pathology. Lymphogranulomatosis, or Hodgkin's disease, affects people of all age groups with equal frequency. In the structure of morbidity among young people (15 - 30 years), 50% of confirmed cases are attributed to the male and female population. The second peak of morbidity is observed among older persons (over 45 years), among whom about 70% of patients are men.

Causes of the occurrence Etiological factors of the disease have not been reliably determined, but there are data on the increased incidence among residents of certain regions, which allows one to draw conclusions about the effect of viruses and environmental factors on the incidence rate. Among predisposing factors distinguish: Hereditary predisposition (the presence of relatives with the disease of lymphoid tissue); Congenital and acquired immunodeficiency; The presence of autoimmune diseases (see lupus erythematosus: symptoms, treatment, rheumatoid arthritis: symptoms and treatment). There is a close association of infectious mononucleosis caused by the Epstein-Barr virus, with the development of lymphogranulomatosis. However, this disease does not occur in every carrier of the virus. According to the studies, the isolated effect of this factor determines the probability of occurrence of lymphogranulomatosis in only 0.1%. In this case, the Epstein-Barr virus is detected in 90% of the world's population. People with different immunodeficiency conditions are also at high risk.

Classification The study of foci of pathological growth in granulomatosis made it possible to identify 4 histological types of disease:

  • Granulomatosis with a predominance of lymphoid tissue (5-10%) manifests itself as a limited or widespread uncontrolled growth of lymphocytes (in some cases, together with histiocytes). This type determines the most favorable course and develops without sclerosing and necrosis of the tumor focus;
  • Nodular sclerosis is the most common form of the disease (30-60%) and is manifested by proliferation of connective tissue in the form of nodes. The phase of fibrosis is preceded by the growth of immature and formed lymphoid cells, while the course of the disease is characterized by gradual slow progression;
  • The mixed cell type of granulomatosis (20-40%) is characterized by an unfavorable course and is manifested by multiple foci of fibrosis, often necrotic. The pathological growth of lymphoid tissue is manifested by polymorphic cell proliferation, including lymphocytes, neutrophils, eosinophils and specific Berezovsky-Sternberg cells. Due to the mass death of cells in the foci of fibrosis, this type has a very unfavorable prognosis;
  • With a sharp reduction in the number of lymphocytes or their complete disappearance, the type of lymphogranulomatosis is characterized as lymphoid depletion (less than 5% of cases). This type has two subtypes: diffuse sclerosis, in which the areas of Hodgkin's or Berezovsky-Sternberg cells accumulation and the reticular type, characterized by pathological giant single and multinucleate cells, can occur in the structure of pathological foci of fibrosis. Any of the two subtypes presupposes a severe course of the disease and has an extremely unfavorable prognosis.

Clinical picture of the disease

The first symptoms that the patient notices are enlarged lymph nodes. The onset of the disease is characterized by the appearance of enlarged dense formations under the skin. They are painless to the touch and can occasionally decrease in size, but subsequently increase again. Significant increase and soreness in the area of ​​lymph nodes is noted after drinking alcohol. In some cases it is possible to increase several groups of regional lymph nodes:

  • Cervical and supraclavicular - 60-80% of cases;
  • Lymphonoduses of the mediastinum - 50%.

Along with the local symptoms of the patient, general manifestations (symptoms of group B) are seriously disturbed:

  • Fever lasting more than one week;
  • Abundant perspiration at night (see causes of excessive sweating in women and men);
  • Uncontrolled weight loss (more than 10% of body weight for 6 months)

Symptoms "B" characterize the more severe course of the disease and allow to determine the need for intensive care. Among the other symptoms characteristic of lymphogranulomatosis are:

  • weakness
  • loss of strength
  • deterioration of appetite
  • skin itching
  • cough, chest pain, shortness of breath
  • Abdominal pain, indigestion ascites
  • Pain in the bones

In a number of cases, the only symptom of lymphogranulomatosis for a long time is only a permanent Feeling tired. Problems with breathing occur with an increase in the intrathoracic lymph nodes. As the nodes grow, they gradually squeeze the trachea and cause a persistent cough and other breathing disorders. These symptoms increase in the supine position. In some cases, patients note soreness behind the sternum.