Korean J Pediatr.  2009 Jun;52(6):725-729. 10.3345/kjp.2009.52.6.725.

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

• Baek HJ
• Kook H
• Han DK
• Lee MC
• Jeong TW
• Hwang TJ

• KMID: 2281082
• DOI: http://doi.org/10.3345/kjp.2009.52.6.725

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.

Keyword

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

MeSH Terms

Antibodies, Monoclonal, Murine-Derived
Central Nervous System
Central Nervous System Diseases
Herpesvirus 4, Human
Humans
Lymphohistiocytosis, Hemophagocytic
Stem Cell Transplantation
Stem Cells
Transplants
Rituximab
Antibodies, Monoclonal, Murine-Derived