Blood Res.  2014 Dec;49(4):246-252. 10.5045/br.2014.49.4.246.

Clinical characteristics and treatment outcomes of children with anaplastic large cell lymphoma: a single center experience

Affiliations
  • 1Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. jjseo@amc.seoul.kr

Abstract

BACKGROUND
Anaplastic large cell lymphoma (ALCL) is uncommon in children, accounting for approximately 15% of all cases of childhood non-Hodgkin lymphoma. Despite many studies attempting 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, chemotherapy 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 multi-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 symptoms, lung involvement, and bone marrow involvement were significant prognostic factors 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 subsequently 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.

Keyword

Anaplastic large cell lymphoma; Childhood; Prognosis; Relpase

MeSH Terms

Bone Marrow
Child*
Cohort Studies
Diagnosis
Disease-Free Survival
Drug Therapy
Humans
Lung
Lymph Nodes
Lymphoma, Large-Cell, Anaplastic*
Lymphoma, Non-Hodgkin
Prognosis
Recurrence
Stem Cell Transplantation
T-Lymphocytes

Figure

  • Fig. 1 Overall survival (OS) and event-free survival (EFS) rates of 28 patients with anaplastic large cell lymphoma (A). OS and EFS rates of eight relapsed patients (B).


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