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KMID : 1130320090520060725
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Korean Journal of Pediatrics
2009 Volume.52 No. 6 p.725 ~ p.729
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Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement
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Baek Hee-Jo
Han Dong-Kyun
Hwang Tai-Ju
Kook Hoon
Lee Min-Cheol
Jeong Tae-Woong
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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.
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KEYWORD
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Unrelated stem cell transplantation, Reduced-intensity conditioning, Rituximab, Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, CNS involvement
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