title: Successful unrelated BMT in a patient with Kostmann syndrome complicated by pre-transplant pulmonary 'bacterial' abscesses.
abstract: Kostmann syndrome, severe congenital neutropenia, is often associated with life-threatening bacterial infections. A 5-year-old girl with Kostmann syndrome developed pulmonary abscesses. She was refractory to granulocyte colony-stimulating factor and antibiotics. She underwent unrelated HLA-matched BMT. Myeloablative conditioning consisted of 12-Gy TBI with lung shielding, antithymocyte globulin, etoposide, and cyclophosphamide. After successful engraftment, the pulmonary abscesses resolved by day 75 post-transplant. Although the option of transplantation is not established in the setting of unrelated HLA-matched BMT in Kostmann syndrome, this case may provide useful information. Furthermore, pre-transplant pulmonary bacterial abscesses may not be a contraindication for BMT in some patients with Kostmann syndrome.
authors.affiliation: Department of Pediatrics and Clinical Immunology, Mie University School of Medicine, Tsu, Mie, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Toyoda
authors.forename: E
authors.initials: E
authors.lastname: Azuma
authors.forename: H
authors.initials: H
authors.lastname: Hori
authors.forename: M
authors.initials: M
authors.lastname: Hirayama
authors.forename: M
authors.initials: M
authors.lastname: Kobayashi
authors.forename: K
authors.initials: K
authors.lastname: Isogai
authors.forename: N
authors.initials: N
authors.lastname: Kondo
authors.forename: Y
authors.initials: Y
authors.lastname: Komada
mesh_terms: D016026:Bone Marrow Transplantation / Q000379:methods*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008169:Lung Abscess / Q000150:complications* / Q000382:microbiology*; D016106:Methicillin Resistance; D009503:Neutropenia / Q000150:complications* / Q000151:congenital / Q000628:therapy*; D013203:Staphylococcal Infections / Q000150:complications*; D013577:Syndrome; D019172:Transplantation Conditioning; D014184:Transplantation, Homologous