We reviewed the results of two consecutive United Kingdom Childrens' Cancer Study Group (UKCCSG) studies of children with stage IV Hodgkin's Disease (HD) treated between January 1982 and December 1999. Among 697 children with HD, 67 were diagnosed to be stage IV. The median age at diagnosis was 12.7 years (range 4.4-16.2). Thirty-five (52%) were boys. Thirty-nine patients (58%) had B symptoms at diagnosis. All were treated with 6-8 cycles of ChlVPP chemotherapy regimen (Chlorambucil, Vinblastine, Procarbazine and prednisolone) and only 12 had radiotherapy. The overall survival (OS) at 5 and 10 years was 80.8% and 77.2%, respectively, whilst the event-free survival (EFS) at the same time intervals was 55.2% and 48.8% respectively. Twenty-eight patients (41.79%) relapsed/progressed, 18 (64%) survived after further chemotherapy with or without high-dose therapy and stem cell rescue. Twelve patients died, seven of HD, three from infections and one from secondary acute myeloblastic leukaemia (AML). Although the EFS in this study was lower than other studies, 64% of relapsed patients were salvaged with second-line therapy. It is also anticipated that survivors treated with this non-anthracycline-containing regimen will have less long-term toxicity.
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