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 Jay Pirruccello, Byers W. Shaw, Ronald J. Rubocki, Debra L. Sudan, Alan Norman Langnas, Simon P. Horslen

Research output: Contribution to journalArticle

36 Citations (Scopus)

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 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
JournalBlood
Volume103
Issue number3
DOIs
StatePublished - Feb 1 2004

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Lymphocytes
Liver
Transplantation
Tissue Donors
Paramyxoviridae Infections
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
T-cells
Immune system
Graft vs Host Disease
T-Cell Antigen Receptor
Cytomegalovirus
Granulocytes
Grafts
Immunoglobulin A
Small Intestine
Intestines
Allografts
Immunoglobulin M
Monocytes
Immune System

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency. / Gilroy, Richard K.; Coccia, Peter F.; Talmadge, James E; Hatcher, Lori I.; Pirruccello, Samuel Jay; Shaw, Byers W.; Rubocki, Ronald J.; Sudan, Debra L.; Langnas, Alan Norman; Horslen, Simon P.

In: Blood, Vol. 103, No. 3, 01.02.2004, p. 1171-1174.

Research output: Contribution to journalArticle

Gilroy, Richard K. ; Coccia, Peter F. ; Talmadge, James E ; Hatcher, Lori I. ; Pirruccello, Samuel Jay ; Shaw, Byers W. ; Rubocki, Ronald J. ; Sudan, Debra L. ; Langnas, Alan Norman ; Horslen, Simon P. / Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency. In: Blood. 2004 ; Vol. 103, No. 3. pp. 1171-1174.
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