title: Allogeneic CD34+ -selected peripheral stem cell transplantation from parental donors in children with non-malignant diseases.
abstract: Allogeneic peripheral stem cell transplantation in six children with non-malignant hematologic or metabolic diseases which are eventually fatal was carried out with parental donors. Given three to five HLA mismatches, all grafts underwent CD34+ cell selection as graft-versus-host prophylaxis. The patients received median doses of 16.7 x 10(6) CD34+ cells/kg and 1.2 x 10(4) CD3+ cells/kg. All transplants engrafted. Neutrophils >0.5/nl were reached on day 11 (9-19) and platelets >50/nl on day 13 (10-25). Acute GVHD responding to steriods occured in three of six patients; it was restricted to the skin and overall did not exceed grade I. Two patients died of viral infections and four are alive with stable blood counts for 13, 15, 25 and 26 months. For children with non-malignant diseases which will eventually be fatal and which can be cured or ameliorated by allogeneic BMT, CD34+-selected stem cell transplants from mismatched or even haploidentical parents can be used if no other suitable donor is available. With high CD34+ cell doses and low CD3+ cell numbers, engraftment and avoidance of acute GVHD can be expected. Infections after transplantation remain the primary threat to survival.
authors.affiliation: Department of Pediatric Hematology-Oncology and Endocrinology, West German Tumor Center, University Hospital, Essen, Germany.
authors.forename: B
authors.initials: B
authors.lastname: Kremens
authors.forename: O
authors.initials: O
authors.lastname: Basu
authors.forename: R
authors.initials: R
authors.lastname: Peceny
authors.forename: H
authors.initials: H
authors.lastname: Grosse-Wilde
authors.forename: U W
authors.initials: UW
authors.lastname: Schaefer
authors.forename: W
authors.initials: W
authors.lastname: Havers
mesh_terms: D000208:Acute Disease; D018952:Antigens, CD34; D001773:Blood Cells / Q000637:transplantation; D001782:Blood Donors; D002423:Cause of Death; D006085:Graft Survival / Q000502:physiology; D006086:Graft vs Host Disease / Q000188:drug therapy; D010290:Parents; D036102:Peripheral Blood Stem Cell Transplantation / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D015996:Survival Rate; D014181:Transplantation Immunology; D014184:Transplantation, Homologous / Q000276:immunology / Q000379:methods / Q000401:mortality
chemical_list: D018952:Antigens, CD34