Anaplastic large cell lymphoma in childhood: analysis of 72 patients treated on The United Kingdom Children's Cancer Study Group chemotherapy regimens

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Abstract
Summary. From June 1990 to June 1998, 72 patients with anaplastic large cell lymphoma (ALCL) were treated with short intensive multi‐agent regimens [non‐Hodgkin's lymphoma (NHL) 9000 and 9602]. Diagnosis was based on morphological and immunophenotypic criteria. Treatment for stage I disease consisted of eight courses (2 × vincristine, doxorubicin, prednisolone; 2 × methotrexate; 2 × cytarabine, thioguanine; and 2 × methotrexate etoposide). For stage II, III and non‐central nervous system (CNS) stage IV, two COPADM (cyclophosphamide, doxorubicin, prednisolone, methotrexate, vincristine), two CYM (cytarabine methotrexate) and a COPADM was given. For CNS‐positive disease, treatment was intensified and contained methotrexate 8 g/m2 and cytarabine 3 g/m2. Fifty‐nine patients (82%) achieved a remission. Thirteen of these relapsed, with a median time to relapse from the start of treatment of 5 months (range 3–14). Relapse included a new site in 9/13 patients. The probabilities of overall and event free survival at 5 years were 65% (53–76%) and 59% (47–70%), respectively, with a median follow up of 4·3 years. Mediastinal and visceral involvement at presentation were found to be predictive of an increased risk of failure.