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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.
Submitted April 16, 2003
Departments of Internal Medicine, Pediatric Hematology/Oncology, Pathology and Microbiology, Surgery, and Pediatrics Gastroenterology, University of Nebraska Medical Center, Omaha, NE, USA * Corresponding author; email: pcoccia{at}unmc.edu.
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 developed full enteral tolerance and normal liver function; and has never had evidence of allograft rejection. After development of mild graft-versus-host disease, studies revealed that over 99% of his CD3+ lymphocytes and 50% of his CD19+ lymphocytes were of donor origin while granulocytes and monocytes remained of recipient origin. He synthesizes polyclonal IgG, IgA, and IgM and has developed antibodies to CMV and parainfluenza III. His T lymphocytes are predominately CD3+CD4-CD8- with T cell receptor
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