Eradication of a dysfunctional HLA-haploidentical T cell system by a second HLA-identical BMT.
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.
Ege
M
M
Department of Paediatrics, University of Ulm, Ulm, Germany.
Manfras
B J
BJ
Barbi
G
G
Schulz
A S
AS
Sigl-Kraetzig
M
M
Debatin
K M
KM
Friedrich
W
W
Mueller
S M
SM
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