What is Lymphogranulomatosis in Children?
Lymphogranulomatosis in children is a malignant disease in which painless chronic swelling of the lymph nodes occurs in one area of the child’s body or in several.
Lymphogranulomatosis is diagnosed in children under 6 and from 6 to 16 years old. But most often, the disease affects adults, not children. The risk of getting sick is higher in boys.
Causes of Lymphogranulomatosis in Children
The causes of lymphogranulomatosis in children by science have not yet been established. Researchers believe that the disease can be caused by an oncogenic virus, but the viral cause of the disease has not been proven.
Pathogenesis during Lymphogranulomatosis in Children
The pathogenesis of lymphogranulomatosis consists in the progressive reproduction of the descendants of the mutant cell and their metastasis along the lymphatic and circulatory pathways. The disease in most cases develops at a slow pace over several years. Basically, patients have a decrease in immunity or a decrease in the number of T-lymphocytes in the blood.
At the beginning of the disease, reactive “inflammation” of the sinuses of the lymph node occurs, later reticular cells multiply focal, giant forms appear (the so-called Berezovsky-Sternberg cells), which have 4-6 hyperchromic nuclei and a light cytoplasm.
Around these cell forms are lymphocytes, histiocytes, eosinophils, neutrophils. This is a typical picture of granuloma, where the name of the disease comes from. The pathognomonic morphological sign of lymphogranulomatosis is the presence of granulomas and “lubrication” of the normal structure of the lymph node.
Depending on the ratio of the types of cells in the granuloma and the degree of proliferation of connective tissue in the lymph node, 4 histological forms of lymphogranulomatosis are distinguished:
- scleronodular (or nodular sclerosis),
- lymphoid,
- mixed cell,
- reticular (or a form of lymphoid depletion).
Symptoms of Lymphogranulomatosis in Children
The disease begins unnoticed. At first, a sick child feels absolutely normal. He finds on the neck an enlarged, painless, dense texture lymph node. Less often, mediastinal nodes, inguinal or axillary, are the first to react. Even less often, lymphogranulomatosis in children manifests itself with an isolated lesion of the stomach, spleen, lungs, intestines, bones, bone marrow.
With the abdominal form of granulomatosis, which in principle is very rare, the retroperitoneal lymph nodes are first affected. On palpation, any nodes do not cause pain. At first they have a densely elastic consistency, are not soldered between themselves and the skin. Gradually, they become inactive due to adhesions, condense, however, not reaching a degree of rocky density and never soldering to the skin.
A common symptom of granulomatosis in children is damage to the spleen. But for palpation, it is available in about a third of cases. An important symptom is an increase in body temperature. At first, the fever is episodic, then it occurs in waves, does not pass when taking acetylsalicylic acid, antibiotics, analgin. Along with fever, the child develops sweating, especially at night. But chills are not observed. Feverish syndrome is observed in 60 children with a given diagnosis of 100.
In a third of cases, the disease begins with itching of the skin, which is becoming more persistent, it can not be removed by conventional means. Children may complain of headache, stiffness in joints and muscles, palpitations. At the beginning of the disease, as a rule, the child loses body weight, he develops weakness, and there may be a decrease in appetite. But these symptoms are not called specific. In some cases, hepatolienal syndrome also occurs.
Immunity is impaired, because bacterial and viral infections often join. In most children, the composition is changed: relative or absolute lymphocytopenia, neutrophilic leukocytosis with a moderate nuclear-nuclear shift, increased ESR. At the beginning of the disease, platelets are normal. There may be eosinophilia, normochromic anemia.
In the terminal stage of lymphogranulomatosis, symptoms of general intoxication are more pronounced, the function of the cardiovascular, respiratory, and nervous systems is impaired. With modern methods of treatment, the patient can live 60-80 months from the onset of the disease. But there are reports of cases of complete recovery from lymphogranulomatosis, when adequate chemo- and radiation therapy was carried out.
At the end, lymphogranulomatosis degenerates into sarcoma in some cases, which aggravates the patient’s condition. Anemia and exhaustion are progressing rapidly, with thrombocytopenia and bleeding.
The abdominal form of lymphogranulomatosis in children is difficult. The child has the following symptoms:
- heavy sweat
- abdominal pain
- high fever
- leukopenia
- high ESR
- sharp shift of the blood formula to the left
A poor prognostic sign is liver damage. The best prognosis is given in cases where ESR is not sharply increased and the level of lymphocytes is sufficient. unfavorable prognosis in almost all cases of children’s illness.
Two forms of the disease according to the speed of the clinical course:
- chronic
- subacute
Forms of non-generalized lymphogranulomatosis in children:
- local
- regional
In case of local damage, 1-2 adjacent regions are affected, while in regional case, 2 or more lymph nodes of non-adjacent zones are involved on one side of the diaphragm. The generalized stage is characterized by an increase in 2 or more groups of lymph nodes located on both sides of the diaphragm, as well as involvement in the spleen process.
Laboratory signs of intoxication:
- lymphocytopenia
- ESR increase of more than 30 mm per hour
- increase in the content of C-reactive protein
- increase in the content of alpha-2 and gamma globulins, sialic acids
- increase in ceruloplasmin, fibrinogen
Diagnosis of Lymphogranulomatosis in Children
If the enlarged lymph nodes are located on the neck, in the area of the mediastinum or armpits, then the diagnosis of lymphogranulomatosis is simplified. It is necessary to distinguish the disease in question from others, in which the lymph nodes are also enlarged. With lymphadenitis, palpation of the nodes causes pain. In infectious mononucleosis and leukemia, a characteristic blood picture is observed. Tuberculous lymphoma fuses with the skin. Lymphosarcoma quickly goes beyond the glandular capsule, grows rapidly, but a biopsy is necessary for an accurate diagnosis.
Diagnosis in cases of abdominal lymphogranulomatosis is difficult. General symptoms appear, but doctors cannot determine their cause for a long time until the pressure felt by the patient or palpation of the abdominal lymph nodes reinforce suspicions of lymphogranulomatosis. In this case, it is necessary to clarify the diagnosis by laparotomy.
Treatment of Lymphogranulomatosis in Children
Treatment of lymphogranulomatosis in children depends on the current stage of the disease. The best results are noted with an increase in one lymph node in one area. This gland is removed by surgical methods, and then this area is irradiated.
When several lymph nodes are affected, high-dose irradiation of the affected area is shown, which is carried out with a cobalt gun, which allows you to spare the skin. In addition to cobalt irradiation, combination therapy with cytostatic drugs is also proposed. Five two-week treatment cycles are carried out with two-week breaks between the individual cycles.
Effective drugs:
- natulan,
- vincristine,
- bleomycin,
- prednisone.
Prevention of Lymphogranulomatosis in children
Children put on dispensary observation. In the first two years, you need to visit a hematologist once every 3 months, and then once every 6 months, since most relapses are recorded precisely during the first 2 years.
Prevention of lymphogranulomatosis consists in reducing the effect of mutagens on the body (chemicals, radiation, UV rays). Sanitation of infectious foci and hardening are of great importance in the preventive plan. Fluorography is sometimes useful.