title: Split chimerism after allogeneic bone marrow transplantation in Chediak-Higashi syndrome.
abstract: Chediak-Higashi Syndrome (CHS) is a hereditary multiorgan disease associated with a lymphoproliferative disorder termed 'accelerated phase' (AP). As AP is often life-threatening, hematopoietic stem cell transplantation has been proposed as the only curative treatment for CHS. Here, we report a 1-year-old Japanese boy with CHS who received an HLA-matched unrelated BMT at the AP stage, which resulted in split chimerism. We evaluated the chimerism status of isolated leukocytes and found that only a limited population of T and NK cells was of donor origin and the majority of these and other hematopoietic cells was of host origin. Clinical outcome was successful, and the patient is currently alive and well, free of AP and serious infections more than 18 months after BMT.
authors.affiliation: Department of Pediatrics, Yokohama City University School of Medicine, Yokohama, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Yamazaki
authors.forename: H
authors.initials: H
authors.lastname: Takahashi
authors.forename: H
authors.initials: H
authors.lastname: Fujii
authors.forename: T
authors.initials: T
authors.lastname: Miyamae
authors.forename: M
authors.initials: M
authors.lastname: Mori
authors.forename: K
authors.initials: K
authors.lastname: Fujioka
authors.forename: T
authors.initials: T
authors.lastname: Funabiki
authors.forename: S
authors.initials: S
authors.lastname: Yokota
authors.forename: N
authors.initials: N
authors.lastname: Arai
authors.forename: K
authors.initials: K
authors.lastname: Ikuta
mesh_terms: D000740:Anemia / Q000209:etiology; D016026:Bone Marrow Transplantation / Q000276:immunology*; D002609:Chediak-Higashi Syndrome / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D013163:Splenomegaly / Q000209:etiology; D013921:Thrombocytopenia / Q000209:etiology; D018183:Transplantation Chimera / Q000276:immunology*; D014184:Transplantation, Homologous / Q000276:immunology*; D016896:Treatment Outcome