title: Unrelated bone marrow transplantation in a Wiskott-Aldrich syndrome patient sharing two HLA-extended haplotypes with the donor.
abstract: We report a case of BMT from an unrelated donor (MUD) in a patient affected by Wiskott- Aldrich syndrome (WAS). The donor-recipient pair was completely identical for two HLA-extended haplotypes. The conditioning regimen consisted of Bu 14 mg/kg followed by CY 200 mg/kg. GVHD prophylaxis was carried out with CsA plus short-term MTX. Allogeneic engraftment was obtained without any signs of acute or chronic GVHD. At fifteen months from the transplant the patient was in an excellent clinical condition. This case confirms the role of BMT from MUD in WAS, and suggests that complete donor-recipient identity for two entire HLA-extended haplotypes is a particularly favorable immunogenetical condition in this type of transplant.
authors.affiliation: Istituto di Clinica Medica, Cattedra di Genetica Medica, Centro Trapianti Midollo Osseo, Università di Cagliari, Italy.
authors.forename: G
authors.initials: G
authors.lastname: La Nasa
authors.forename: A
authors.initials: A
authors.lastname: Pizzati
authors.forename: A
authors.initials: A
authors.lastname: Ledda
authors.forename: A
authors.initials: A
authors.lastname: Vacca
authors.forename: M
authors.initials: M
authors.lastname: Arras
authors.forename: L
authors.initials: L
authors.lastname: Contu
mesh_terms: D000328:Adult; D016026:Bone Marrow Transplantation / Q000276:immunology*; D002675:Child, Preschool; D005260:Female; D006680:HLA Antigens / Q000235:genetics*; D006239:Haplotypes; D006801:Humans; D008297:Male; D014019:Tissue Donors; D014923:Wiskott-Aldrich Syndrome / Q000235:genetics / Q000276:immunology / Q000628:therapy*
chemical_list: D006680:HLA Antigens