title: Eradication of a dysfunctional HLA-haploidentical T cell system by a second HLA-identical BMT.
abstract: A 12-year-old boy treated for SCID at 1 month of age by HLA-haploidentical BMT developed a lymphoproliferative disease of unknown etiology at the age of 9 years characterized by sustained, marked elevation of circulating CD8+ donor T cells and by diffuse infiltration of the liver by CD8+ T cells. Because of progressive liver disease, the patient underwent a second BMT from a younger HLA-matched sister. This treatment induced an effective graft-versus-graft reaction and led to complete replacement of the HLA-nonidentical, dysfunctional T cell system, resolution of the hepatopathy and full reconstitution of T and B cell functions.
authors.affiliation: Department of Paediatrics, University of Ulm, Ulm, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Ege
authors.forename: B J
authors.initials: BJ
authors.lastname: Manfras
authors.forename: G
authors.initials: G
authors.lastname: Barbi
authors.forename: A S
authors.initials: AS
authors.lastname: Schulz
authors.forename: M
authors.initials: M
authors.lastname: Sigl-Kraetzig
authors.forename: K M
authors.initials: KM
authors.lastname: Debatin
authors.forename: W
authors.initials: W
authors.lastname: Friedrich
authors.forename: S M
authors.initials: SM
authors.lastname: Mueller
mesh_terms: D016026:Bone Marrow Transplantation; D018414:CD8-Positive T-Lymphocytes / Q000473:pathology; D002648:Child; D006239:Haplotypes; D006650:Histocompatibility Testing; D006801:Humans; D008107:Liver Diseases / Q000209:etiology / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000209:etiology / Q000235:genetics*; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000628:therapy; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D018183:Transplantation Chimera; D014184:Transplantation, Homologous / Q000009:adverse effects*; D014185:Transplantation, Isogeneic