Important note: Information in this article was accurate in 1987. The state of the art may have changed since the publication date.
MALIGNANT T CELL AND OTHER NON-HODGKIN'S LYMPHOMA OF CHILDHOOD
Cancer in Children. Clinical Management. Second Edition. Voute PA et al, eds. Current Treatment of Cancer, New York, Springer-Verlag, p. 152-63, 1986.. Unique Identifier : AIDSLINE ICDB/87637337 Amylon MD; Link MP; Murphy SB; Stanford Univ. Medical Center, Children's Hosp. at Stanford,; Stanford, CA 94305
Abstract:
The epidemiology, etiology, clinical presentation, staging and prognosis, tumor biology, and treatment of T cell lymphoblastic, lymphoblastic non-T cell, large cell (histiocytic), and other rare non-Hodgkin's lymphomas in children are discussed with emphasis on the first of these entities. Maturational stages of normal and malignant T cells and their reactions to antigens and results of modern treatment regimens for lymphoblastic lymphoma are described. Lymphoblastic lymphoma is rare in very young children; however, the incidence increases throughout childhood. There is a strong male predominance. The general principles of therapy are similar to those which have been applied in childhood acute lymphoblastic leukemia (ALL). Remission induction is a necessary first step. Current induction regimens, which usually contain vincristine and prednisone with cyclophosphamide and/or an anthracycline, successfully induce complete remission in 90-100% of patients with localized disease and 85-95% of patients with advanced stage lymphoblastic lymphomas. To prevent relapse, consolidation chemotherapy is usually given, often including L-asparaginase. Prophylactic therapy of the CNS is necessary in advanced stage patients, and intrathecal medication in induction and continuation without cranial radiotherapy is effective. Recent reports indicate that patients with localized lymphoma have an 80-100% chance for long-term, disease-free survival. Non-T lymphoblastic lymphomas may be treated most appropriately with chemotherapy regimens that are effective in childhood ALL, and the prognosis depends on stage. For large cell lymphoma, it generally appears that the principles of therapy being successfully applied to lymphoblastic lymphoma will be quite effective. (68 Refs)
Keywords: Antineoplastic Agents, Combined/*THERAPEUTIC USE Cell Transformation, Neoplastic/PATHOLOGY Child Human HTLV-BLV Infections/DRUG THERAPY Leukemia, Lymphocytic/DRUG THERAPY Lymphoma, Large-Cell/DRUG THERAPY Lymphoma, Non-Hodgkin's/*DRUG THERAPY/PATHOLOGY Neoplasm Staging Remission Induction *T-Lymphocytes/PATHOLOGY MONOGRAPH
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