title: High-dose cytosine arabinoside and fractionated total body irradiation as a preparative regimen for the treatment of children with acute lymphoblastic leukemia and Down syndrome by allogeneic bone marrow transplantation.
abstract: The early toxicity, incidence of graft-versus-host disease (GVHD) and long-term follow-up were evaluated in two children with Down syndrome (DS) treated for acute lymphoblastic leukemia (ALL) in second complete remission by HLA-matched sibling allogeneic bone marrow transplantation (BMT). Preparative conditioning therapy consisted of cytosine arabinoside (Ara-C) and fractionated total body irradiation (F-TBI) and GVHD prophylaxis of cyclosporin A. The conditioning regimen was well tolerated, the only acute complication being mild mucositis. Engraftment (polymorphonuclear cells >500/microliter) was documented by day +17 in both patients. One child remains in continuous complete remission, without medical problems, 60 months after BMT. The second patient died from complications associated with chronic GVHD 21 months following BMT. Ara-C and F-TBI is a well-tolerated preparative regimen for children with DS undergoing allogeneic BMT.
authors.affiliation: Pediatric Hematology Department, University of Milan, S Gerardo Hospital, Monza, Italy.
authors.forename: V
authors.initials: V
authors.lastname: Conter
authors.forename: P
authors.initials: P
authors.lastname: D'Angelo
authors.forename: C
authors.initials: C
authors.lastname: Rizzari
authors.forename: M
authors.initials: M
authors.lastname: Jankovic
authors.forename: C
authors.initials: C
authors.lastname: Dampier
authors.forename: G
authors.initials: G
authors.lastname: Masera
authors.forename: F L
authors.initials: FL
authors.lastname: Johnson
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D002675:Child, Preschool; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D003561:Cytarabine; D003630:Daunorubicin / Q000008:administration & dosage; D004314:Down Syndrome / Q000150:complications*; D017809:Fatal Outcome; D006086:Graft vs Host Disease / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D008297:Male; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D011241:Prednisone / Q000008:administration & dosage; D016879:Salvage Therapy; D014184:Transplantation, Homologous; D014750:Vincristine / Q000008:administration & dosage; D014916:Whole-Body Irradiation
chemical_list: D003561:Cytarabine; D014750:Vincristine; D015122:Mercaptopurine; D011241:Prednisone; D008727:Methotrexate; D003630:Daunorubicin