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Blood, 1 February 2004, Vol. 103, No. 3, pp. 1171-1174.
Prepublished online as a Blood First Edition Paper on October 2, 2003; DOI 10.1182/blood-2003-04-1187.


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TRANSPLANTATION
Brief report

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, Jr, Ronald J. Rubocki, Debra L. Sudan, Alan N. Langnas, and Simon P. Horslen

From the Departments of Internal Medicine, Pediatric Hematology/Oncology, Pathology and Microbiology, Surgery, and Pediatric Gastroenterology, University of Nebraska Medical Center, Omaha.

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 {gamma}{delta} heterodimers and CD3+CD4-CD8+ with CD8{alpha}{alpha} 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. (Blood. 2004;103:1171-1174)


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