title: Successful unrelated cord blood transplantation using a reduced-intensity conditioning regimen in a 6-month-old infant with congenital neutropenia complicated by severe pneumonia.
abstract: Here we report the first successful unrelated cord blood transplantation (CBT) using reduced-intensity conditioning for the 'treatment of congenital neutropenia in a 6-month-old infant with complications of severe pneumonia probably due to Staphylococcus aureus infection. Because the patient showed no response to treatment with granulocyte colony-stimulating factor and had a cytogenetic aberration, unrelated CBT with an HLA-DRB1 genotypic mismatch was performed. The number of infused cells was 15 x 10(7)/kg. The preparative regimen was fludarabine, cyclophosphamide, and 6 Gy of total body irradiation. Teicoplanin was administered for bacterial pneumonia. Neutrophil engraftment was achieved on day 41 and was followed by clinical improvement. The patient gradually caught up on growth and development after the CBT. Unrelated CBT using a reduced-intensity conditioning regimen may be an effective treatment for congenital neutropenia.
authors.affiliation: Department of Pediatrics, Reconstructive Medicine and Tissue Engineering, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Yozo
authors.initials: Y
authors.lastname: Nakazawa
authors.forename: Kazuo
authors.initials: K
authors.lastname: Sakashita
authors.forename: Mizuho
authors.initials: M
authors.lastname: Kinoshita
authors.forename: Ken
authors.initials: K
authors.lastname: Saida
authors.forename: Tomonari
authors.initials: T
authors.lastname: Shigemura
authors.forename: Ryu
authors.initials: R
authors.lastname: Yanagisawa
authors.forename: Naoto
authors.initials: N
authors.lastname: Shikama
authors.forename: Takehiko
authors.initials: T
authors.lastname: Kamijo
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
mesh_terms: D001827:Body Height; D036101:Cord Blood Stem Cell Transplantation / Q000379:methods*; D006085:Graft Survival / Q000276:immunology; D006684:HLA-DR Antigens / Q000235:genetics / Q000276:immunology; D059811:HLA-DRB1 Chains; D006410:Hematopoiesis; D006648:Histocompatibility; D006801:Humans; D007223:Infant; D008297:Male; D009503:Neutropenia / Q000150:complications / Q000151:congenital / Q000628:therapy*; D011014:Pneumonia / Q000188:drug therapy / Q000209:etiology*; D012074:Remission Induction / Q000379:methods; D014019:Tissue Donors; D018183:Transplantation Chimera; D019172:Transplantation Conditioning / Q000379:methods*
chemical_list: D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains