Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Gilroy, RK; Coccia, PF; Talmadge, JE; Hatcher, LI; Pirruccello, SJ; Shaw, BW; Rubocki, RJ; Sudan, DL; Langnas, AN; Horslen, SP

Published Date

  • February 1, 2004

Published In

Volume / Issue

  • 103 / 3

Start / End Page

  • 1171 - 1174

PubMed ID

  • 14525785

International Standard Serial Number (ISSN)

  • 0006-4971

Digital Object Identifier (DOI)

  • 10.1182/blood-2003-04-1187


  • eng

Conference Location

  • United States