title: CD-34 selected hematopoetic stem cell transplantation from HLA identical family members for fanconi anemia.
abstract: Hematopoetic stem cell transplantation, even from an HLA 6/6 identical family member is associated with an increased frequency of complication in fanconi anemia (FA). The increased susceptibility for chromosomal breaks has been suggested as a contributory factor for increased risk of toxicity, graft versus host disease (GVHD) and increased incidence of post-transplant solid tumors. Therefore, non-irradiation based preparative regimens usually containing fludarabine and T-cell depletion of HLA geno-identical bone marrow cells have increasingly been used in patients with FA. Here, we report three children with FA who underwent CD-34 selected HSCT from HLA-identical family donors with reduced intensity fludarabine-based regimen.
authors.affiliation: Department of Pediatric Hematology, Hacettepe University School of Medicine, Ankara, Turkey.
authors.forename: Yasemin Isik
authors.initials: YI
authors.lastname: Balci
authors.forename: Yasemin
authors.initials: Y
authors.lastname: Akdemir
authors.forename: Fatma
authors.initials: F
authors.lastname: Gumruk
authors.forename: Mualla
authors.initials: M
authors.lastname: Cetin
authors.forename: Fikret
authors.initials: F
authors.lastname: Arpaci
authors.forename: Duygu
authors.initials: D
authors.lastname: Uckan
mesh_terms: D000293:Adolescent; D018952:Antigens, CD34 / Q000378:metabolism*; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D004248:DNA (Cytosine-5-)-Methyltransferases / Q000037:antagonists & inhibitors; D005199:Fanconi Anemia / Q000276:immunology / Q000628:therapy*; D005260:Female; D006086:Graft vs Host Disease; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006680:HLA Antigens / Q000378:metabolism*; D018380:Hematopoietic Stem Cell Transplantation; D006650:Histocompatibility Testing; D006801:Humans; D008212:Lymphocyte Depletion; D008297:Male; D011994:Recombinant Proteins; D013601:T-Lymphocytes / Q000276:immunology; D019172:Transplantation Conditioning; D014740:Vidarabine / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D018952:Antigens, CD34; D000970:Antineoplastic Agents; D006680:HLA Antigens; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D004248:DNA (Cytosine-5-)-Methyltransferases; D014740:Vidarabine; C024352:fludarabine