Successful umbilical cord blood transplantation from an unrelated donor for a patient with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

Citation
M. Minegishi et al., Successful umbilical cord blood transplantation from an unrelated donor for a patient with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, BONE MAR TR, 27(8), 2001, pp. 883-886
Citations number
22
Language
INGLESE
art.tipo
Editorial Material
Categorie Soggetti
Hematology,"Medical Research Diagnosis & Treatment
Journal title
BONE MARROW TRANSPLANTATION
ISSN journal
0268-3369 → ACNP
Volume
27
Issue
8
Year of publication
2001
Pages
883 - 886
Database
ISI
SICI code
0268-3369(200104)27:8<883:SUCBTF>2.0.ZU;2-D
Abstract
We report a case of a 5-year-old girl with EBV-associated hemophagocytic ly mphohistiocytosis (EBV-HLH) who underwent cord blood (CB) stem cell transpl antation (CBSCT) from an unrelated donor. The patient presented with persis tent high-grade fever and hepatosplenomegaly, Because the disease was refra ctory to immunochemotherapy according to the HLH94 protocol, she received 2 .0 x 10(7) CB nucleated cells/kg body weight (BW) after conditioning with B U/CY/etoposide. No acute GVHD developed, using FK506 for prophylaxis. The n eutrophil count reached >0.5 x 10(9)/l by day 21 and the platelet count rea ched >50 x 10(9)/l by day 84. The patient recovered well with sequelae of n eurological deficits more than 10 months after receiving CBSCT, without sho wing evidence of HLH or chronic GVHD, Real-time PCR proved applicable for e stimation of the EBV load in PBMC of the patient. We conclude that CBSCT ma y be indicated for some cases of refractory EBV-HLH, who have no HLA-matche d siblings and are therefore dependent on unrelated marrow donors.