Clin Pediatr Hematol Oncol.  2013 Oct;20(2):102-107.

Pediatric Extranodal NK/T Cell Lymphoma in a Single Institution

• Ju HY
• Kang HJ
• Hong CR
• Kim SJ
• Lee JW
• Kim H
• Park KD
• Jeon YK
• Kim CW
• Shin HY
• Ahn HS

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.

Keyword

Lymphoma; Extranodal NK-T-Cell; Pediatric; Korea

MeSH Terms

Child
Diagnosis
Disease-Free Survival
Drug Therapy
Follow-Up Studies
Humans
Induction Chemotherapy
Korea
Lymphoma*
Medical Records
Peripheral Blood Stem Cell Transplantation
Prognosis