Korean Journal of Pediatrics 2009;52(6):725-729.
Published online June 15, 2009.
Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement
Hee Jo Baek1, Hoon Kook1, Dong Kyun Han1, Min-Cheol Lee1, Tae Woong Jeong2, Tai Ju Hwang1
1Departments of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
2Departments of Radiology, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
저강도 전처치와 rituximab 후 타인 조혈모세포 이식을 시행한 중추신경계를 침범한 Epstein-Barr 바이러스 관련 혈구포식 림프조직구증
백희조1, 국훈1, 한동균1, 이민철1, 정태웅2, 황태주1
1전남대학교 의과대학 화순전남대학교병원 소아과학교실
2전남대학교 의과대학 화순전남대학교병원 영상의학과교실
Correspondence: 
Hoon Kook, Email: hoonkook@chonnam.ac.kr
Abstract
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and might be an effective option in patients with EBV-HLH with progressive CNS disease. Moreover, the addition of rituximab to RIC appears to be safe and effective in suppressing EBV in the patients with EBV-HLH.
Key Words: Unrelated stem cell transplantation, Reduced-intensity conditioning, Rituximab, Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, CNS involvement


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