title: Allogeneic bone marrow transplantation in a child with hemoglobinopathy olmsted.
abstract: The authors describe the first successful bone marrow transplant for the treatment of hemoglobinopathy Olmsted in a boy who presented with severe transfusion-dependent hemolytic anemia and jaundice at age 4 months. He received bone marrow from an HLA-identical sibling with normal hemoglobin electrophoresis after conditioning with busulfan, cyclophosphamide, and antithymocyte globulin when he was 18 months old. The posttransplant course was uneventful. Two years after transplantation the patient has a normal hemoglobin level without evidence of hemolysis. DNA analysis shows 100% chimerism of donor cell origin, confirming full engraftment with normal hematopoietic cells.
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Graz School of Medicine, Austria. christian.urban@kfunigraz.ac.at
authors.forename: Christian
authors.initials: C
authors.lastname: Urban
authors.forename: Karl-Heinz
authors.initials: KH
authors.lastname: Preisegger
authors.forename: Walter
authors.initials: W
authors.lastname: Krugluger
authors.forename: Pierre
authors.initials: P
authors.lastname: Hopmeier
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Schwinger
authors.forename: Herwig
authors.initials: H
authors.lastname: Lackner
authors.forename: Reinhold
authors.initials: R
authors.lastname: Kerbl
authors.forename: Hans-Jürgen
authors.initials: HJ
authors.lastname: Dornbusch
authors.forename: Martin
authors.initials: M
authors.lastname: Benesch
mesh_terms: D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D002066:Busulfan / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D017931:DNA Primers / Q000737:chemistry; D005914:Globins / Q000235:genetics; D006453:Hemoglobinopathies / Q000235:genetics / Q000628:therapy*; D006455:Hemoglobins, Abnormal / Q000235:genetics*; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D016133:Polymerase Chain Reaction; D019172:Transplantation Conditioning; D014184:Transplantation, Homologous
chemical_list: D018906:Antineoplastic Agents, Alkylating; D017931:DNA Primers; D006455:Hemoglobins, Abnormal; C098095:hemoglobin Olmsted; D003520:Cyclophosphamide; D005914:Globins; D002066:Busulfan