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Hodgkin's lymphoma (lymphogranulomatosis) in children
Last reviewed: 23.11.2021
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Hodgkin's lymphoma in children (Hodgkin's disease, lymphogranulomatosis) is a malignant tumor of lymphoid tissue that has a specific granulomatous histological structure. The disease occurs in all age groups, except for children of the first year of life; at the age of 5 years is rare. Among all the lymphomas in children, Hodgkin's disease is about 40%. In the age group up to 12 years, boys are more likely to get sick, in teenagers the ratio of sick boys and girls is approximately the same. The incidence of children with lymphogranulomatosis, according to the International Registers, is 0.7-0.9 cases per 100 000 children. The risk of developing Hodgkin's lymphoma is higher in children with primary immunodeficiency (ataxia-telangiectasia, agamma-globulinemia), rheumatoid arthritis and systemic lupus erythematosus. In Ukraine, there is no correct incidence of lymphogranulomatosis.
[Causes of the hodgkin's lymphoma (lymphogranulomatosis)
Causes of Hodgkin's Lymphoma in Children
The cause of Hodgkin's lymphoma in children is unknown. The role of the Epstein-Barr virus detected in tumor cells with lymphogranulomatosis is discussed (most often in mixed-cell variant in children of younger age group). A hypothesis of the pathogenesis of Hodgkin's lymphoma is suggested, according to which the block of programmed death, apoptosis, lies at the basis of uncontrolled proliferation of tumor cells formed in the lymph node as a result of the mutation of germic B-lymphocytes.
Symptoms of the hodgkin's lymphoma (lymphogranulomatosis)
Symptoms of Hodgkin's Lymphoma in Children
The main symptom of Hodgkin's lymphoma in a child is an increase in lymph nodes (lymphadenopathy).
Among extranodular localizations, it is necessary to note (up to a quarter of cases) the defeat of the spleen, and pleura and lungs are often involved in the process. Possible damage to any organs - bones, skin, liver, bone marrow. Very rarely observed tumor germination in the spinal cord, specific infiltration of the kidney and thyroid gland.
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Diagnostics of the hodgkin's lymphoma (lymphogranulomatosis)
Diagnosis of lymphogranulomatosis in children
In a clinical blood test for Hodgkin's lymphoma, moderate neutrophilia and lymphopenia are detected, almost all patients show an increase in ESR. Moderate eosinophilia and thrombocytosis are possible (these changes are not directly diagnostic).
There are no specific changes in biochemical blood analysis. The activity of lactate dehydrogenase is not increased or increased by more than 2 times compared to normal values (a greater increase in activity is possible with hemolysis). An increase in the concentration of ferritin, ceruloplasmin and fibrinogen has no diagnostic significance, but in some clinics these factors are taken into account as prognostic factors.
Diagnosis of lymphogranulomatosis
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Treatment of the hodgkin's lymphoma (lymphogranulomatosis)
Treatment of Hodgkin's lymphoma in children
In different countries, numerous programs for the treatment of Hodgkin's lymphoma have been developed. Their principal elements are radiation therapy and polychemotherapy using a relatively narrow range of drugs. It is possible to use only radiotherapy, only chemotherapy or a combination of both methods. Radiotherapy and chemotherapy programs for lymphogranulomatosis are constantly being improved: their effectiveness increases, immediate and late toxicity decreases without development of resistance. The tactics of Hodgkin's lymphoma therapy are determined by the stage of the disease and the age of the patient.
Forecast
Forecast for Hodgkin's disease in children
Hodgkin's disease in children has a different prognosis, it, first of all, depends on the stage at which the treatment is started. When local forms of lymphogranulomatosis (IA, IIA) complete recovery is possible in 70-80% of children, although a complete remission is achieved in 90%. You can talk about recovery only 10 years after the successful course of primary treatment. Most relapses occur within the first 3-4 years after the end of therapy.