All issues > Volume 52(6); 2009

Case Report

Korean J Pediatr. 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 HJ Baek¹, Hoon H Kook¹, Dong Kyun DK Han¹, Min-Cheol MC Lee¹, Tae Woong TW Jeong², Tai Ju TJ 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

Correspondence Hoon H 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.

Keywords :Unrelated stem cell transplantation, Reduced-intensity conditioning, Rituximab, Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, CNS involvement