Pediatric Extranodal NK/T Cell Lymphoma in a Single Institution
- Author:
Hee Young JU
1
;
Hyoung Jin KANG
;
Che Ry HONG
;
Sung Jin KIM
;
Ji Won LEE
;
Hyeri KIM
;
Kyung Duk PARK
;
Yoon Kyung JEON
;
Chul Woo KIM
;
Hee Young SHIN
;
Hyo Seop AHN
Author Information
1. Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea. hyshin@snu.ac.kr
- Publication Type:Original Article
- Keywords:
Lymphoma;
Extranodal NK-T-Cell;
Pediatric;
Korea
- MeSH:
Child;
Diagnosis;
Disease-Free Survival;
Drug Therapy;
Follow-Up Studies;
Humans;
Induction Chemotherapy;
Korea;
Lymphoma;
Medical Records;
Peripheral Blood Stem Cell Transplantation;
Prognosis
- From:Clinical Pediatric Hematology-Oncology
2013;20(2):102-107
- CountryRepublic of Korea
- Language:English
-
Abstract:
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 investigate 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 within one month of diagnosis; thus, five patients underwent chemotherapy including L-asparaginase, and one patient underwent chemotherapy without L-asparaginase. All patients 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 significant 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.
