Pediatric Extranodal NK/T Cell Lymphoma in a Single Institution / 임상소아혈액종양

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.