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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 (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 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 Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted September 8, 2003 Accepted February 25, 2004 Postnatal Thymus Transplantation with Immunosuppression as Treatment for DiGeorge Syndrome M L 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 Pediatrics, Duke University Medical Center, Durham, NC, USA; Immunology, Duke University Medical Center, Durham, NC, USA Pediatrics, Duke University Medical Center, Durham, NC, USA Immunology, Duke University Medical Center, Durham, NC, USA Medicine, Duke University Medical Center, Durham, NC, USA; Human Vaccine Institute, Duke University Medical Center, Durham, NC, USA Pathology, Duke University Medical Center, Durham, NC, USA Surgery, Duke University Medical Center, Durham, NC, USA * Corresponding author; email: Mary L Markert/Rankin/MedSch/mc/Duke{at}mc. Complete DiGeorge syndrome is a fatal congenital disorder characterized by athymia. The goal of this study was to assess if immune suppression with rabbit anti-thymocyte globulin followed by postnatal thymus transplantation would lead to T cell function in six patients who had T cells at the time of transplantation. All infants had fewer than 50 recent thymic emigrants (CD3+CD45RA+CD62L+)/cubic millimeter (cumm) and some proliferative response to the mitogen phytohemagglutinin. Four of the infants had rash, lymphadenopathy and, oligoclonal populations of T cells in the periphery. Five of six patients survive with follow up of 11 - 26 months. The one death was secondary to respiratory syncytial virus infection. The five surviving patients developed a mean of 764 CD3+ T cells/cumm (range 587 - 1061/cumm), a mean of 236 recent thymic emigrants (range 129-507/cumm), and normal proliferative responses to phytohemaglutinin (follow up from day 244 to 614). The TCR repertoire normalized in patients presenting with oligoclonal T cells. All patients had evidence of thymopoiesis on allograft biopsy. Postnatal thymus transplantation after rabbit anti-thymocyte globulin shows promise as therapy for infants with complete DiGeorge syndrome who have significant proliferative responses to mitogens or who present with 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: J D M Edgar T cell immunodeficiency J. Clin. Pathol., September 1, 2008; 61(9): 988 - 993. [Abstract] [Full Text] [PDF] 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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