• Title/Summary/Keyword: Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

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Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement (저강도 전처치와 rituximab 후 타인 조혈모세포 이식을 시행한 중추신경계를 침범한 Epstein-Barr 바이러스 관련 혈구포식 림프조직구증)

  • Baek, Hee Jo;Kook, Hoon;Han, Dong Kyun;Lee, Min-Cheol;Jeong, Tae Woong;Hwang, Tai Ju
    • Clinical and Experimental Pediatrics
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    • v.52 no.6
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    • pp.725-729
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    • 2009
  • 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.