title: [Long-term remission in an acute monoblastic leukemia patient with down syndrome after cord blood transplantation].
abstract: A 4-year-old boy with Down syndrome (DS) was diagnosed as having acute monoblastic leukemia (AML-M5a). Leukemic cells were CD33+, CD56+ and CD4+, with t(9;11) on cytogenetic analysis and MLL gene rearrangement. After 2 courses of induction therapy using an AML 99-Down protocol failed to obtain complete remission, the patient received cord blood transplantation from an HLA-matched donor (CBT) following a conditioning regimen comprising total body irradiation and cyclophosphamide. Only cyclosporin A was used for graft-versus-host disease prophylaxis. Stem cell transplantation may not be indicated for AML patient with DS in first remission, who display a high rate of life-threatening and fatal toxicity on therapy. This patient remained well controlled in complete remission for 4 years, representing a rare case of DS with chemotherapy-resistant AML successfully treated with a CBT.
authors.affiliation: Department of Pediatrics, Dokkyo University School of Medicine.
authors.forename: Hidemitsu
authors.initials: H
authors.lastname: Kurosawa
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Tsuboi
authors.forename: Hagane
authors.initials: H
authors.lastname: Shimaoka
authors.forename: Mayuko
authors.initials: M
authors.lastname: Okuya
authors.forename: Daisuke
authors.initials: D
authors.lastname: Nakajima
authors.forename: Takayuki
authors.initials: T
authors.lastname: Matsunaga
authors.forename: Susumu
authors.initials: S
authors.lastname: Hagisawa
authors.forename: Yuya
authors.initials: Y
authors.lastname: Sato
authors.forename: Kenichi
authors.initials: K
authors.lastname: Sugita
authors.forename: Mitsuoki
authors.initials: M
authors.lastname: Eguchi
mesh_terms: D002675:Child, Preschool; D036101:Cord Blood Stem Cell Transplantation; D016572:Cyclosporine / Q000008:administration & dosage; D004314:Down Syndrome / Q000150:complications*; D006086:Graft vs Host Disease / Q000517:prevention & control; D006801:Humans; D007948:Leukemia, Monocytic, Acute / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D008297:Male; D012074:Remission Induction; D013997:Time Factors; D019172:Transplantation Conditioning; D016896:Treatment Outcome
chemical_list: D016572:Cyclosporine