FAVORABLE OUTCOME AFTER 1-YEAR TREATMENT OF CHILDHOOD T-CELL LYMPHOMAT-CELL ACUTE LYMPHOBLASTIC-LEUKEMIA

Background. For T-malignancies in children a poor prognosis is reporte d. In these malignancies a combination of lymphoma and leukemia is com monly seen at presentation and most patients are treated according to protocols for acute lymphoblastic leukemia (ALL). These protocols are often designed for the majority of ALL cases, i.e., progenitor-B-ALL. In pediatric lymphoblastic non-Hodgkin's lymphoma without bone marrow infiltration various protocols have been used. The most frequently rep orted regimens show variable survival rates between 40 and 75%. Patien ts and Methods. From 1989 we have treated 32 consecutive patients with T-cell malignancies, irrespective of localization, with a protocol co nsisting of a 4-agent induction treatment followed by high doses of me thotrexate, and cytosine-arabinoside and intensified bleomycin, adriam ycin, cyclophosphamide, vincristin, prednisone (BACOP) courses. Treatm ent duration for each patient was 1 year. Twenty-one of the 32 patient s had stage IV disease. Follow-up ranged from 1.6 to 7.6 years (median 4.2 years). Results. Overall event-free survival (EFS) was 72%, while in those with stage IV disease it was 67%. No therapy-related deaths occurred. Neither stage, initial leukocyte value, mediastinal involvem ent, bone marrow involvement, nor the presence of CD1, CD3, CD4, CD8, or CD10 epitopes was prognostically significant. Evaluation of toxicit y revealed a minimal decrease of carbon monoxide diffusion and cardiac shortening fraction. Conclusion. A relatively short but intensive che motherapy can be used in T-cell malignancies. The EFS is satisfying, b ut larger studies are needed. (C) 1998 Wiley-Liss, Inc.