title: Successful unrelated cord blood transplantation for chronic granulomatous disease: a case report and review of the literature.
abstract: CGD is a rare inherited immunodeficiency disorder that is caused by disability of oxidative killing. We presented a two-yr-old boy with CGD who was suffering from multiple systemic abscesses. He received the first BMT from his HLA-haploidentical mother after conditioning with Flu, melphalan, and ATG. Although the maximum of 42% donor chimerism was achieved, it disappeared 73 days after the BMT. Then, we performed 5/6-matched unrelated cord blood re-transplantation after conditioning with Flu, Bu, and TBI (2 Gy). Engraftment and complete donor chimerism were achieved on days 18 and 19, respectively. The patient is now free from infection and maintains complete donor chimerism without GVHD 36 months after the cord blood re-transplantation. We postulate that the unrelated CBT has a potential to be an alternative strategy and might be beneficial for patients with CGD who do not have an HLA-identical donor.
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan. mochi-k@fmu.ac.jp
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Mochizuki
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kikuta
authors.forename: Masaki
authors.initials: M
authors.lastname: Ito
authors.forename: Mitsuko
authors.initials: M
authors.lastname: Akaihata
authors.forename: Hideki
authors.initials: H
authors.lastname: Sano
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Ohto
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Hosoya
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002675:Child, Preschool; D036101:Cord Blood Stem Cell Transplantation; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000628:therapy*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007239:Infections / Q000209:etiology / Q000628:therapy; D008297:Male; D012008:Recurrence; D012086:Reoperation; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents