title: Successful umbilical cord blood transplantation from an unrelated donor for a patient with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
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 x 10(7) 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 x 10(9)/l by day 21 and the platelet count reached >50 x 10(9)/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.
authors.affiliation: Department of Pediatric Oncology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Minegishi
authors.forename: Y
authors.initials: Y
authors.lastname: Ohashi
authors.forename: S
authors.initials: S
authors.lastname: Kumaki
authors.forename: Y
authors.initials: Y
authors.lastname: Sasahara
authors.forename: T
authors.initials: T
authors.lastname: Hayashi
authors.forename: H
authors.initials: H
authors.lastname: Asada
authors.forename: T
authors.initials: T
authors.lastname: Okuyama
authors.forename: I
authors.initials: I
authors.lastname: Hakozaki
authors.forename: T
authors.initials: T
authors.lastname: Sato
authors.forename: S
authors.initials: S
authors.lastname: Tsuchiya
mesh_terms: D001782:Blood Donors; D002675:Child, Preschool; D004279:DNA, Viral / Q000097:blood; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D005312:Fetal Blood / Q000166:cytology; D018380:Hematopoietic Stem Cell Transplantation; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology / Q000628:therapy* / Q000821:virology; D006801:Humans; D016133:Polymerase Chain Reaction; D019562:Viral Load
chemical_list: D004279:DNA, Viral