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KMID : 0387820130200020102
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Clinical Pediatric Hematology-Oncology
2013 Volume.20 No. 2 p.102 ~ p.107
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Pediatric Extranodal NK/T Cell Lymphoma in a Single Institution
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Ju Hee-Young
Kang Hyoung-Jin
Hong Che-Ry
Kim Sung-Jin
Lee Ji-Won
Kim Hye-Ri
Park Kyung-Duk
Jeon Yoon-Kyung
Kim Chul-Woo
Shin Hee-Young
Ahn Hyo-Seop
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Abstract
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Background: Extranodal NK/T cell lymphoma (ENKTL) is extremely rare in children, and there have been few reports on pediatric ENKTL. The purpose of this study was to inves-tigate the clinical features and treatment outcomes of pediatric ENKTL.
Methods: The study involved a review of the medical records of eight pediatric patients who were diagnosed with ENKTL.
Results: Among the eight patients, three were in stage I of the disease, and five were in stages II to IV. The median follow-up period was 90.8 months. Two stage I patients were nasal type, and the other six patients were non-nasal type. Two patients died with-in one month of diagnosis; thus, five patients underwent chemotherapy including L-as-paraginase, and one patient underwent chemotherapy without L-asparaginase. All pa-tients showed an overall response after induction chemotherapy, with four showing a complete response (CR) and two showing a partial response (PR). Two newly diagnosed patients and one relapsed patient underwent autologous peripheral blood stem cell transplantation (aPBSCT). The five-year overall survival (OS) rate was 50%, and the five-year progression-free survival (PFS) rate was 46.9%. Ann Arbor stage was a sig-nificant prognostic factor for OS (P=0.042).
Conclusion: Advanced-stage pediatric ENKTL was associated with a grave prognosis. However, intensive chemotherapy with L-asparaginase resulted in an overall response, and aPBSCT could be beneficial for pediatric ENKTL.
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KEYWORD
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Lymphoma, Extranodal NK-T-Cell, Pediatric, Korea
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