title: Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency.
abstract: The syndrome of multiple intestinal atresia with immunodeficiency is a rare, invariably fatal congenital disorder. At 16 months of age, a child with this syndrome underwent liver-small bowel transplantation from a 1-of-6 HLA-matched donor. He acquired full enteral tolerance and normal liver function and has never shown evidence of allograft rejection. After mild graft-versus-host disease developed, studies revealed that more than 99% of his CD3(+) lymphocytes and 50% of his CD19(+) lymphocytes were of donor origin, whereas granulocytes and monocytes remained of recipient origin. He synthesizes polyclonal immunoglobulin G (IgG), IgA, and IgM and has developed antibodies to cytomegalovirus (CMV) and parainfluenza 3. His T lymphocytes are predominately CD3(+)CD4(-)CD8(-) with T-cell receptor gammadelta heterodimers and CD3(+)CD4(-)CD8(+) with CD8alphaalpha homodimers, populations consistent with an intraepithelial lymphocyte phenotypic profile. We postulate that he has engrafted a donor intestine-derived immune system and is incapable of rejecting his engrafted organs.
authors.affiliation: Department of Pediatric Hematology/Oncology, University of Nebraska Medical Center, 982168 Nebraska Medical Center, Omaha, NE 68198-2168, USA.
authors.forename: Richard K
authors.initials: RK
authors.lastname: Gilroy
authors.forename: Peter F
authors.initials: PF
authors.lastname: Coccia
authors.forename: James E
authors.initials: JE
authors.lastname: Talmadge
authors.forename: Lori I
authors.initials: LI
authors.lastname: Hatcher
authors.forename: Samuel J
authors.initials: SJ
authors.lastname: Pirruccello
authors.forename: Byers W
authors.initials: BW
authors.lastname: Shaw
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Rubocki
authors.forename: Debra L
authors.initials: DL
authors.lastname: Sudan
authors.forename: Alan N
authors.initials: AN
authors.lastname: Langnas
authors.forename: Simon P
authors.initials: SP
authors.lastname: Horslen
mesh_terms: D003586:Cytomegalovirus Infections / Q000209:etiology; D006085:Graft Survival; D006086:Graft vs Host Disease / Q000209:etiology; D006801:Humans; D007136:Immunoglobulins / Q000096:biosynthesis; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000601:surgery*; D007223:Infant; D007409:Intestinal Atresia / Q000276:immunology / Q000601:surgery*; D007421:Intestine, Small / Q000637:transplantation*; D016031:Liver Transplantation; D016131:Lymphocyte Subsets / Q000276:immunology; D008297:Male; D014019:Tissue Donors
chemical_list: D007136:Immunoglobulins