Document Detail


Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency.
MedLine Citation:
PMID:  14525785     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
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
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2003-10-02
Journal Detail:
Title:  Blood     Volume:  103     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  2004 Feb 
Date Detail:
Created Date:  2004-01-19     Completed Date:  2004-03-10     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1171-4     Citation Subset:  AIM; IM    
Affiliation:
Department of Pediatric Hematology/Oncology, University of Nebraska Medical Center, 982168 Nebraska Medical Center, Omaha, NE 68198-2168, USA.
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MeSH Terms
Descriptor/Qualifier:
Cytomegalovirus Infections / etiology
Graft Survival
Graft vs Host Disease / etiology
Humans
Immunoglobulins / biosynthesis
Immunologic Deficiency Syndromes / immunology,  surgery*
Infant
Intestinal Atresia / immunology,  surgery*
Intestine, Small / transplantation*
Liver Transplantation*
Lymphocyte Subsets / immunology
Male
Tissue Donors
Chemical
Reg. No./Substance:
0/Immunoglobulins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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