Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement.
10.3345/kjp.2009.52.6.725
- Author:
Hee Jo BAEK
1
;
Hoon KOOK
;
Dong Kyun HAN
;
Min Cheol LEE
;
Tae Woong JEONG
;
Tai Ju HWANG
Author Information
1. Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea. hoonkook@chonnam.ac.kr
- Publication Type:Case Report
- Keywords:
Unrelated stem cell transplantation;
Reduced-intensity conditioning;
Rituximab;
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis;
CNS involvement
- MeSH:
Antibodies, Monoclonal, Murine-Derived;
Central Nervous System;
Central Nervous System Diseases;
Herpesvirus 4, Human;
Humans;
Lymphohistiocytosis, Hemophagocytic;
Stem Cell Transplantation;
Stem Cells;
Transplants;
Rituximab
- From:Korean Journal of Pediatrics
2009;52(6):725-729
- CountryRepublic of Korea
- Language:English
-
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.
