Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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저강도 전처치와 rituximab 후 타인 조혈모세포 이식을 시행한 중추신경계를 침범한 Epstein-Barr 바이러스 관련 혈구포식 림프조직구증

Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement

  • 백희조 (전남대학교 의과대학 화순전남대학교병원 소아과학교실) ;
  • 국훈 (전남대학교 의과대학 화순전남대학교병원 소아과학교실) ;
  • 한동균 (전남대학교 의과대학 화순전남대학교병원 소아과학교실) ;
  • 이민철 (전남대학교 의과대학 화순전남대학교병원 병리학교실) ;
  • 정태웅 (전남대학교 의과대학 화순전남대학교병원 영상의학과교실) ;
  • 황태주 (전남대학교 의과대학 화순전남대학교병원 소아과학교실)
  • Baek, Hee Jo (Department of Pediatrics, Chonnam National University Hwasun Hospital) ;
  • Kook, Hoon (Department of Pediatrics, Chonnam National University Hwasun Hospital) ;
  • Han, Dong Kyun (Department of Pediatrics, Chonnam National University Hwasun Hospital) ;
  • Lee, Min-Cheol (Department of Pathology, Chonnam National University Hwasun Hospital) ;
  • Jeong, Tae Woong (Department of Radiology, Chonnam National University Hwasun Hospital) ;
  • Hwang, Tai Ju (Department of Pediatrics, Chonnam National University Hwasun Hospital)
  • 투고 : 2009.05.26
  • 심사 : 2009.06.04
  • 발행 : 2009.06.15
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초록

중추신경계의 침범을 동반한 Epstein-Barr 바이러스 관련 혈구포식 림프조직구증은 조혈모세포이식을 시행하지 않으면 예후가 좋지 않다. 또한, 고식적인 강도의 전처치시 이식관련 사망률이 높다. 따라서 저자들은 진행성 중추신경계 침범을 보인 Epstein-Barr 바이러스 관련 혈구포식 림프조직구증 2례에서 저강도 전처치와 rituximab 후 타인 조혈모세포이식을 시행하여 치료하였기에 이를 보고하고자 한다.

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.

키워드

참고문헌

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피인용 문헌

  1. Hemophagocytic lymphohistiocytosis diagnosed by brain biopsy vol.58, pp.9, 2015, https://doi.org/10.3345/kjp.2015.58.9.358