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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.
TRANSPLANTATION Donor immune reconstitution after liversmall bowel transplantation for multiple intestinal atresia with immunodeficiencyFrom 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
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