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

Richard K. Gilroy, Peter F. Coccia, James E. Talmadge, Lori I. Hatcher, Samuel J. Pirruccello, Byers W. Shaw, Ronald J. Rubocki, Debra L. Sudan, Alan N. Langnas, Simon P. Horslen

Research output: Contribution to journalArticle

36 Scopus citations


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 γδ heterodimers and CD3+CD4 -CD8+ with CD8αα 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.

Original languageEnglish (US)
Pages (from-to)1171-1174
Number of pages4
Issue number3
StatePublished - Feb 1 2004


ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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