Treatment Outcome for Anaplastic Large Cell Lymphoma in children : A Single Center Experience

Treatment Outcome for Anaplastic Large Cell Lymphoma in children : A Single Center Experience

Treatment Outcome for Anaplastic Large Cell Lymphoma in children : A Single Center Experience

(구연):
Release Date : 2013. 10. 18(금)
Jee Yeon Han, Jin Kyung Suh, Kyung Nam Koh, Ho Joon Im, Jong Jin Seo
Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, Korea
한지연, 서진경, 고경남, 임호준, 서종진
울산의대 서울아산병원 소아청소년과 소아혈액종양분과

Abstract

Background : Anaplastic large cell lymphoma(ALCL) is uncommon in children accounting for approximately 15% of all childhood non-Hodgkin’s lymphoma. Chemotherapy regimens for pediatric ALCL vary. Despite many studies attempting new treatment strategies and evaluating the effects of each treatment, the outcome of patients with ALCL has not significantly improved during the last decades, and optimal treatment for pediatric ALCL is not yet established. Patients & Methods : From July 1998 to April 2013, newly diagnosed ALCL patients in our institute were reviewed. We evaluated general characteristics of patients, chemotherapy regimens and overall survival rate, event free survival rate as the outcomes of treatment. Results : Twenty-eight patients with ALCL (male 64%, female 36%) were eligible for our study. The median age at diagnosis was 10.8. Lymph-node involvement was the most common presentation (78%). Of extranodal disease, mediastinal involvement was the most frequent (35%), followed by visceral (21%), bone (14%), lung (14%), bone marrow (14%) involvement. In 16 patients, ALK study was performed; 12 had positive results and the other 6 had negative results. CCG-5941, an aggressive multi-agent T-cell lineage chemotherapy regimen was used the most for the treatment of ALCL in our institute (57%). The 2-year and the 5-year overall survival were 92% and 87.2%. The 2-year event-free survival and the 5-year event-free survival were 79.2% and 70.1%. There were 7 relapses and 3 deaths. All of 3 deaths were attributed to sepsis during chemotherapy for refractory disease after relapse. 3 of 7 relapses were treated by high-dose chemotherapy and autologous stem cell transplantation (HDCT-ASCT). One was alive without relapse after chemotherapy and 2 had second relapses after HDCT-ASCT. These 2 had allogenic stem cell transplantation after second relapse and one of them was treated by brentuximab after allogenic SCT. All 3 patients treated by stem cell transplantation are still alive. The 5-year event-free survival had significant difference by stage (p-value 0.02). Conclusions : Our study showed that children with ALCL showed favorable outcome with multi-agent chemotherapy, and relapsed patients could be salvaged with high-dose chemotherapy with HDCT-ASCT or allogeneic SCT. A prospective study is warranted to define the optimal treatment for pediatric ALCL.

Keywords: Anaplastic large cell lymphoma, ,