Results of a uniform treatment protocol based on the Fab/Lmb96 study for B cell non-hodgkin lymphoma in children and adolescents of Hong Kong

Abstract

PURPOSE/OBJECTIVE: Survival has steadily improved for mature childhood B cell non-Hodgkin lymphoma (NHL) using short intensive treatment regimens. We aim to review the results of a uniform territory-wide treatment protocol for B cell NHL established in Hong Kong since 1998. MATERIALS AND METHODS: The five hospitals of Hong Kong forming the Hong Kong Paediatric Haematology and Oncology Study Group (HKPHOSG) established a uniform treatment protocol based on the FAB/LMB96 study to treat children and adolescents with B cell NHL since 1998. The clinical data of all cases diagnosed between 1-Jan-1998 and 31-Dec-2011 were reviewed up to the date of 31-Dec-2012. RESULTS: During the study period, a total of 55 cases including 44 with Burkitt lymphoma (BL) and 11 with diffuse large B cell lymphoma (DLBCL) were diagnosed. Six cases of primary mediastinal large B cell lymphomas were excluded. Forty-two (76%) were males. The median age was 7.7 years (range, 2.6 to 17.8 years). The median follow up duration was 5.8 years (range, 0.11 to 16.1 years). The Murphy stages consisted of 13% stage 1 (n = 7), 25% stage 2 (n = 14), 31% stage 3 (n = 17) and 31% stage 4 (n = 17). Six cases (11%) had CNS involvement. Treatment groups were stratified as 11% group A (n = 6), 65% group B (n = 36) and 24% group C (n = 13; 7 CNS-; 6 CNS+). The 5-year event-free survival (EFS) and overall survival (S) were 80.9% and 84.8%, with those for BL 79.1% and 81.4% and for DLBCL 87.5% and 100%, respectively. The 5-year EFS and S for CNS- cases were 84.7% and 87.0% and those for CNS+ cases 50.0% and 66.7%, respectively (p ? 0.041 for CNS+ v CNS-). CONCLUSIONS: The treatment results for all stages of the CNS- B cell NHL are significantly better than those for the CNS+ cases. Future goal should aim at improving the survival of the CNS+ B cell NHL.