Results of a uniform treatment protocol based on the ALCL99 study for anaplastic large cell lymphoma in children and adolescents of Hong Kong

Abstract

PURPOSE/OBJECTIVE: We aim to review the results of a uniform territory-wide treatment protocol for anaplastic large cell lymphoma (ALCL) established in Hong Kong since 2000. 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 ALCL99 study to treat children and adolescents with ALCL since 2000. The clinical data of all cases diagnosed between 1-Jan-2000 and 31-Dec-2011 were reviewed up to the date of 31-Dec-2012. RESULTS: During the study period, a total of 13 cases of ALCL were diagnosed. Eight (62%) were males. The median age was 9.4 years (range, 6.3 to 15.4 years). The median follow up duration was 5.73 years (range, 0.35 to 12.6 years). The Murphy stages consisted of 7.5% stage 1 (n = 1), 23% stage 2 (n = 3), 62% stage 3 (n = 8) and 7.5% stage 4 (n ? 1; bone marrow involvement). The sites of lymphoma included nodal and/or extranodal sites such as lung, pericardium, mediastinum, bone and skin. Two cases had skin nodules only. None had CNS involvement. Ten cases were ALK+ , one case was ALK- and 2 cases had unknown ALK status. Three cases were CD3+. Treatment groups were stratified as 46% standard risk (n = 6) and 54% high risk (n = 7). The 5-year eventfree survival (EFS) and overall survival (S) were 68.4% and 84.6%, respectively. All cases except one achieved CR1. One case died of refractory disease. Three cases had disease relapse of which 2 cases could be salvaged with stem cell transplantation and/or vinblastine treatment. CONCLUSIONS: Although EFS for ALCL was relatively low at <70%, overall survival of >80% could be achieved with salvage therapy. Future goal should aim at finding the optimal therapy for this rare lymphoma type through inter-group cooperative trials.