| Unrelated stem cell transplantation after reduced-intensity
conditioning plus rituximab for Epstein-Barr virus-associated
hemophagocytic lymphohistiocytosis with CNS involvement |
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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 |
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백희조1, 국훈1, 한동균1, 이민철1, 정태웅2, 황태주1 |
1전남대학교 의과대학 화순전남대학교병원 소아과학교실
2전남대학교 의과대학 화순전남대학교병원 영상의학과교실 |
Correspondence:
Hoon Kook, Email: hoonkook@chonnam.ac.kr
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| Abstract |
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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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