Results of treatment of lymphoblastic lymphoma in children and adolescents of Hong Kong

Abstract

PURPOSE/OBJECTIVE: Lymphoblastic lymphoma is one of the major subtypes of childhood non-Hodgkin lymphoma. We aim to review the treatment results of T cell and B cell lymphoblastic lymphoma in children and adolescents of Hong Kong. MATERIALS AND METHODS: The five hospitals forming the Hong Kong Paediatric Haematology and Oncology Study Group (HKPHOSG) treated children and adolescents with lymphoblastic lymphoma using successive treatment protocols for acute lymphoblastic leukaemia from 1995-2008 and a protocol based on EURO-LB02 study from 2008. The clinical data of all cases diagnosed between 1-Mar-1995 and 31-Dec-2011 were reviewed up to the date of 31-Dec-2012. RESULTS: During the study period, a total of 42 patients including 34 T cell (T-LBL) and 8 B cell (B-LBL) cases were diagnosed. Thirty-one (74%) were males. The median age was 10.4 years (range, 3.0 to 17.7 years). The median follow up duration was 7.0 years (range, 0.08 to 17.5 years). The Murphy stages consisted of 7% stage 1 (n ? 3), 10% stage 2 (n ? 4), 43% stage 3 (n ? 18) and 40% stage 4 (n ? 17). The 5-year event-free survival (EFS) and overall survival (S) were 66.3% and 76.7%, with those for T-LBL 60.3% and 73.7% and for B-LBL 87.5% and 87.5%, respectively. The 5-year EFS and S for stage 1 and 2 cases were both 100% whilst those for stage 3 were 76.9% and 72.3% and for stage 4 47.1% and 70.6%, respectively (p = 0.038 for stage 4 v stage 1-2). All 3 cases with primary refractory disease died. Only 3 of 9 cases with relapsed disease could be salvaged with second line chemotherapy followed by allogeneic stem cell transplantation. CONCLUSIONS: Although survival of LBL with contemporary treatment reaches >70%, stage 3 or 4 lymphoblastic lymphoma which is refractory to treatment or relapses has very poor prognosis.