Clinical Characteristics and Treatment Outcomes of Non-anaplastic Peripheral T-Cell Lymphoma in Children and Adolescents: A Single-center Experience / 임상소아혈액종양

ABSTRACT

BACKGROUND: Peripheral T-cell lymphoma (PTCL) is a rare form of non-Hodgkin's lymphoma (NHL), and it is usually associated with poor outcome. Here, we report our experience in treating this disease over 19 years, with the aim of helping to establish better treatment methods.METHODS: We retrospectively investigated 18 non-anaplastic PTCL cases that were diagnosed at a medical center in Seoul between October 1995 and October 2014. The clinical characteristics, treatments, and outcomes were reviewed.RESULTS: According to the World Health Organization (WHO) classifications for PTCL, 11 patients had PTCL, not otherwise specified (PTCL-NOS), 6 patients had extranodal natural killer/T-cell lymphoma, nasal type (ENKL), and 1 patient had subcutaneous panniculitis-like T-cell lymphoma. Patients were treated with various chemotherapeutic regimens. Of these 18 patients, 5 (27.7%) relapsed and 7 (38.9%) died from disease progression. Two relapsed patients received high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HDCT-ASCT). The 5-year event-free and overall survival rates were 43.2% and 66.7% in all cases, 45.5% and 54.5% in PTCL-NOS, and, 25.0% and 83.3% in ENKL, respectively.CONCLUSION: PTCL-NOS showed a suboptimal outcome. Among 6 ENKL patients, 3 relapsed, but 2 of 3 relapsed patients were salvaged. For better prognosis, HDCT-ASCT in relapsing and refractory PTCL and chemo-radiotherapy in ENKL could be considered as a salvage treatment. Larger studies are needed to confirm the outcome. Furthermore, an effort should be made to develop more efficient initial therapies through collaborative research.