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KMID : 0366220140490040246
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Korean Journal of Hematology
2014 Volume.49 No. 4 p.246 ~ p.252
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Clinical characteristics and treatment outcomes of children with anaplastic large cell lymphoma: a single center experience
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Han Jee-Yeon
Suh Jin-Kyung
Lee Seong-Wook
Koh Kyung-Nam
Im Ho-Joon
Seo Jong-JIn
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Abstract
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Background: Anaplastic large cell lymphoma (ALCL) is uncommon in children, accounting for approx-imately 15% of all cases of childhood non-Hodgkin lymphoma. Despite many studies at-tempting new treatment strategies, treatment outcomes have not significantly improved, and the optimal treatment for pediatric ALCL has not been established.
Methods: The records of newly diagnosed ALCL patients at our institute between July 1998 and April 2013 were reviewed. We evaluated the general characteristics of the patients, chemo-therapy regimens, overall survival (OS) rates, and event-free survival (EFS) rates.
Results: Twenty-eight ALCL patients were eligible. The median age at diagnosis was 10.8 years. Lymph node involvement was the most common presentation (79%). CCG-5941, a mul-ti-agent T-cell lineage chemotherapy, was the predominant treatment regimen (57%). The five-year OS and EFS rates were 88% and 69%, respectively. Stage, the presence of B symp-toms, lung involvement, and bone marrow involvement were significant prognostic fac-tors for EFS (P=0.02, 0.01, 0.01, and 0.02, respectively). Eight patients relapsed, and three died during the study period. Four of the eight patients who relapsed were treated with high-dose chemotherapy and autologous stem cell transplantation (HDCT-ASCT). Two of the four who had undergone HDCT-ASCT developed secondary relapses and were sub-sequently treated with allogeneic SCT or brentuximab.
Conclusion: We found that treatment outcomes with multi-agent chemotherapy in children with ALCL were similar to those of previous reports, and that relapsed patients could be salvaged with HDCT-ASCT or allogeneic SCT. A prospective, larger cohort study is warranted to define the optimal treatment for pediatric ALCL.
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KEYWORD
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Anaplastic large cell lymphoma, Childhood, Prognosis, Relpase
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