ChlVPP chemotherapy in children with stage IV Hodgkin’s disease: results of the UKCCSG HD 8201 and HD 9201 studies Ayad Atra, 1 Elizabeth Higgs, 2 Michael Capra, 3 Ann Elsworth, 4 John Imeson, 4 Martin Radford 5 and Martin Hewitt 3 on behalf of the UKCCSG/Hodgkin’s Disease Group 1 Department of Paediatrics, Riyadh Armed Forces Hospital, Riyadh Kingdom of Saudi Arabia, 2 Department of Paediatric Oncology, St James’s University Hospital, Leeds, 3 Queen’s Medical Centre, Nottingham, 4 UKCCSG, University of Leicester, Department of Epidemiology and Public Health, Leicester, and 5 Southampton General Hospital, Department of Child Health, Southampton, UK Received 31 January 2002; accepted for publication 17 June 2002 Summary. 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 mye- loblastic 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. Keywords: ChlVPP, children, stage IV, advanced Hodgkin’s disease. Various chemotherapy regimens with or without involved field radiation have been used in the treatment of children with stage IV Hodgkin’s Disease (HD). MOPP (Nitrogen mustard, Vincristine, Procarbazine and prednisone), first introduced in 1964 (Longo et al, 1986), was the gold standard treatment until 1973 when the ABVD (Doxoru- bicin, Bleomycin, Vinbastine and Dacarbazine) regimen was introduced. The latter regimen has produced better results and less toxicity (Santoro et al, 1987). Hybrid regimens, such as MOPP/ABVD, MOPP/ABV and others, have pro- duced results comparable to ABVD alone (Radford et al, 1995; Viviani et al, 1996; Connors et al, 1997; Glick et al, 1998). Patients are usually given 6–8 cycles of chemother- apy or two additional cycles after the confirmation of complete remission (CR). The recommended treatment of children with stage IV HD in the UK during the 1980s and 1990s was 6–8 cycles of ChlVPP (Chlorambucil, Vinblastine, Procarbazine and prednisolone). Radiotherapy was not routinely used because of concerns about long-term toxicity and also because its role in stage IV disease remains unclear. The aims of this study were to assess the overall survival (OS) and event-free survival (EFS) of children with stage IV HD treated with this non-anthracycline-containing regimen and to review the relapse pattern and salvage rate after second-line therapy. PATIENTS AND METHODS Six hundred and ninety-seven eligible patients were entered on the United Kingdom Children Cancer Study Group (UKCCSG) HD studies, HD 8201 between January 1982 and June 1992, and HD 9201 between July 1992 and December 1999. Eighty-nine patients were diagnosed to have stage IV HD (Ann Arbor Staging System) by the treating centres. Twenty-two were excluded from analysis as they either received a different chemotherapy regimen (n ¼ 8), were not confirmed to be stage IV (n ¼ 11) or not confirmed to have HD (n ¼ 3) when reviewed centrally. The remaining Correspondence: Dr A. Atra, E 245, Department of Paediatrics, Riyadh Armed Forces Hospital, PO Box 7897, Riyadh 11159, Kingdom of Saudi Arabia. E-mail: ayadatra@hotmail.com British Journal of Haematology, 2002, 119, 647–651 Ó 2002 Blackwell Publishing Ltd 647