title: Successful HLA-identical sibling cord blood transplantation in a 6-year-old boy with leukocyte adhesion deficiency syndrome.
abstract: A 6-year-old boy with the severe form of the leukocyte adhesion deficiency syndrome (LAD) received a transplant of cord blood (CBT) from his HLA-identical brother. The donor was proved healthy by successful prenatal diagnosis. CBT was performed after conditioning with etoposide, busulfan and cyclophosphamide. After hematopoietic recovery complete chimerism was proved as well as the normal expression of CD11x/CD18 complex on circulating leukocytes. The only post-transplant complication was a mild pneumonitis resolving on the corticosteroid therapy. Thirteen months after CBT the boy is in good health and shows no signs of immunodeficiency. As far as we know this is the first report of successful CBT in a patient with LAD syndrome.
authors.affiliation: 2nd Dept of Pediatrics, University Hospital Motol, Prague, Czech Republic.
authors.forename: J
authors.initials: J
authors.lastname: Starý
authors.forename: J
authors.initials: J
authors.lastname: Bartůnková
authors.forename: P
authors.initials: P
authors.lastname: Kobylka
authors.forename: V
authors.initials: V
authors.lastname: Vávra
authors.forename: O
authors.initials: O
authors.lastname: Hrusák
authors.forename: P
authors.initials: P
authors.lastname: Calda
authors.forename: V
authors.initials: V
authors.lastname: Král
authors.forename: K
authors.initials: K
authors.lastname: Svorc
mesh_terms: D002648:Child; D002678:Chimera; D017218:Cordocentesis; D005312:Fetal Blood / Q000166:cytology*; D005315:Fetal Diseases / Q000175:diagnosis; D018380:Hematopoietic Stem Cell Transplantation; D006648:Histocompatibility; D006801:Humans; D016130:Immunophenotyping; D007231:Infant, Newborn; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000175:diagnosis / Q000628:therapy*; D008297:Male; D009679:Nuclear Family; D019172:Transplantation Conditioning