학술논문
Successful umbilical cord blood transplantation from an unrelated donor for a patient with Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis.
Document Type
Article
Author
Source
Subject
*EPSTEIN-Barr virus diseases
*CORD blood
*STEM cells
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Language
ISSN
0268-3369
Abstract
We report a case of a 5-year-old girl with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) who underwent cord blood (CB) stem cell transplantation (CBSCT) from an unrelated donor. The patient presented with persistent high-grade fever and hepatosplenomegaly. Because the disease was refractory to immunochemotherapy according to the HLH94 protocol, she received 2.0 × 107 CB nucleated cells/kg body weight (BW) after conditioning with BU/CY/etoposide. No acute GVHD developed, using FK506 for prophylaxis. The neutrophil count reached >0.5 × 109/l by day 21 and the platelet count reached >50 × 109/l by day 84. The patient recovered well with sequelae of neurological deficits more than 10 months after receiving CBSCT, without showing evidence of HLH or chronic GVHD. Real-time PCR proved applicable for estimation of the EBV load in PBMC of the patient. We conclude that CBSCT may be indicated for some cases of refractory EBV-HLH, who have no HLA-matched siblings and are therefore dependent on unrelated marrow donors. Bone Marrow Transplantation (2001) 27, 883–886. [ABSTRACT FROM AUTHOR]