title: Successful related umbilical cord blood transplantation for graft failure following T cell-depleted non-identical bone marrow transplantation in a child with major histocompatibility complex class II deficiency.
abstract: Major histocompatibility complex (MHC) class II deficiency is a rare form of primary combined immunodeficiency that can only be corrected by stem cell transplantation. We report a 4(1/2)-year-old girl with MHC class II deficiency who underwent a related CBT due to graft failure following T cell-depleted non-identical BMT. The patient is alive and well 2 years after the second transplant. A sustained hematopoietic engraftment and a progressive immune recovery have been detected. We conclude that cord blood may be an effective source of hematopoietic stem cells for patients with immuno- deficiency disorders including diseases with a high rate of graft failure.
authors.affiliation: Department of Hematology/Oncology and Bone Marrow Transplant Unit, Hospital de Pediatría 'Prof Dr Juan P Garrahan', Buenos Aires, Argentina.
authors.forename: M
authors.initials: M
authors.lastname: Bonduel
authors.forename: A
authors.initials: A
authors.lastname: Pozo
authors.forename: M
authors.initials: M
authors.lastname: Zelazko
authors.forename: E
authors.initials: E
authors.lastname: Raslawski
authors.forename: S
authors.initials: S
authors.lastname: Delfino
authors.forename: J
authors.initials: J
authors.lastname: Rossi
authors.forename: C
authors.initials: C
authors.lastname: Figueroa
authors.forename: F
authors.initials: F
authors.lastname: Sackmann Muriel
mesh_terms: D016026:Bone Marrow Transplantation; D005260:Female; D005312:Fetal Blood; D006681:HLA-D Antigens / Q000235:genetics*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000628:therapy*; D007223:Infant; D008212:Lymphocyte Depletion; D013601:T-Lymphocytes; D014184:Transplantation, Homologous; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D006681:HLA-D Antigens