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Blood, 15 October 2004, Vol. 104, No. 8, pp. 2574-2581. Prepublished online as a Blood First Edition Paper on April 20, 2004; DOI 10.1182/blood-2003-08-2984. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-08-2984v1 104/8/2574    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Markert, M. L. Articles by Skinner, M. A. Search for Related Content PubMed PubMed Citation Articles by Markert, M. L. Articles by Skinner, M. A. Related Collections Immunobiology Transplantation Immunotherapy Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  TRANSPLANTATION Postnatal thymus transplantation with immunosuppression as treatment for DiGeorge syndrome M. Louise Markert, Marilyn J. Alexieff, Jie Li, Marcella Sarzotti, Daniel A. Ozaki, Blythe H. Devlin, Debra A. Sedlak, Gregory D. Sempowski, Laura P. Hale, Henry E. Rice, Samuel M. Mahaffey, and Michael A. Skinner From the Departments of Pediatrics, Immunology, Medicine, Pathology, and Surgery, and the Human Vaccine Institute, Duke University Medical Center, Durham, NC. Complete DiGeorge syndrome is a fatal congenital disorder characterized by athymia, hypoparathyroidism, and heart defects. Less than half of patients are 22q11 hemizygous. The goal of this study was to assess if immune suppression followed by postnatal thymus transplantation would lead to T-cell function in 6 infant patients who had host T cells at the time of transplantation. All infants had fewer than 50 recent thymic emigrants (CD3+CD45RA+CD62L+) per cubic millimeter (mm3) and all had some proliferative response to the mitogen phytohemagglutinin. Four infants had rash, lymphadenopathy, and oligoclonal populations of T cells in the periphery. Five of 6 patients are alive at the follow-up interval of 15 months to 30 months. The 5 surviving patients developed a mean of 983 host CD3+ T cells/mm3 (range, 536/mm3-1574/mm3), a mean of 437 recent thymic emigrants/mm3 (range, 196/mm3-785/mm3), and normal proliferative responses to phytohemaglutinin (follow-up from day 376 to day 873). The TCR repertoire became polyclonal in patients who presented with oligoclonal T cells. All patients had thymopoiesis on allograft biopsy. Postnatal thymus transplantation after treatment with Thymoglobulin shows promise as therapy for infants with complete DiGeorge syndrome who have significant proliferative responses to mitogens or who develop rash, lymphadenopathy, and oligoclonal T cells. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. L. Markert, J. Li, B. H. Devlin, J. C. Hoehner, H. E. Rice, M. A. Skinner, Y.-J. Li, and L. P. Hale Use of Allograft Biopsies to Assess Thymopoiesis after Thymus Transplantation J. Immunol., May 1, 2008; 180(9): 6354 - 6364. [Abstract] [Full Text] [PDF] W. Li, M. H. Sofi, N. Yeh, S. Sehra, B. P. McCarthy, D. R. Patel, R. R. Brutkiewicz, M. H. Kaplan, and C.-H. Chang Thymic selection pathway regulates the effector function of CD4 T cells J. Exp. Med., September 3, 2007; 204(9): 2145 - 2157. [Abstract] [Full Text] [PDF] M. L. Markert, B. H. Devlin, M. J. Alexieff, J. Li, E. A. McCarthy, S. E. Gupton, I. K. Chinn, L. P. Hale, T. B. Kepler, M. He, et al. Review of 54 patients with complete DiGeorge anomaly enrolled in protocols for thymus transplantation: outcome of 44 consecutive transplants Blood, May 15, 2007; 109(10): 4539 - 4547. [Abstract] [Full Text] [PDF] B. M. Ogle, L. J. West, D. J. Driscoll, S. E. Strome, R. R. Razonable, C. V. Paya, M. Cascalho, and J. L. Platt Effacing of the T Cell Compartment by Cardiac Transplantation in Infancy J. Immunol., February 1, 2006; 176(3): 1962 - 1967. [Abstract] [Full Text] [PDF] S. Al-Tamemi, B. Mazer, D. Mitchell, P. Albuquerque, A. M. V. Duncan, C. McCusker, and N. Jabado Complete DiGeorge Anomaly in the Absence of Neonatal Hypocalcemia and Velofacial and Cardiac Defects Pediatrics, September 1, 2005; 116(3): e457 - e460. [Abstract] [Full Text] [PDF]

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