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Blood, 1 March 2004, Vol. 103, No. 5, pp. 1625-1631. Prepublished online as a Blood First Edition Paper on November 6, 2003; DOI 10.1182/blood-2003-07-2525. This Article Full Text (PDF) All Versions of this Article: 2003-07-2525v1 103/5/1625    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 Malbran, A. Articles by de Bracco, M. M Search for Related Content PubMed PubMed Citation Articles by Malbran, A. Articles by de Bracco, M. M Social Bookmarking                   What's this? Submitted July 25, 2003 Accepted October 22, 2003 Loss of circulating CD27+ memory B cells and CCR4+ T cells occurring in association with elevated Epstein-Barr virus (ebv) loads in XLP patients surviving primary EBV infection Alejandro Malbran, Liliana Belmonte, Beatriz Ruibal-Ares, Patricia Bare, Ivana Massud, Cecilia Parodi, Marta Felippo, Richard Hodinka, Kathleen Haines, Kim E Nichols, and Maria M de Bracco* Departamento de Alergia e Inmunologia, Hospital Britanico, Buenos Aires, Argentina Division Inmunologia, Instituto de Investigaciones Hematologicas, Academia Nacional de Medicina, Buenos Aires, Argentina Children's Hospital of Philadelphia, Philadelphia, PA, USA * Corresponding author; email: mebracco{at}hematologia.anm.edu.ar. Detailed longitudinal studies of patients with X-linked lymphoproliferative disease (XLP) may increase our understanding of the immunological defects that contribute to development of lymphoma and/or hypogammaglobulinemia in XLP. We describe progressive changes observed in immunoglobulin concentrations, lymphocyte subsets and Epstein Barr virus (EBV) viral loads occurring over a two-year period in a newly infected, but otherwise healthy carrier (patient #9). We compare these findings to those observed in his hypogammaglobulinemic XLP brother (patient #4). IgG, IgM and IgA concentrations increased in patient #9 during acute EBV infection, but thereafter decreased steadily to a level consistent with hypogammaglobulinemia, reaching a plateau 5 months after infection. In both patients, CD19+ B lymphocytes remained below 3% with a contraction of the B cell memory compartment (CD27+ CD19+/CD19+) to 20% (normal range: 32%-56%). T lymphocyte subpopulations showed a reduction in CD4+ T cells and a permanent CD8+ T cell expansion. Interestingly, CXCR3 memory Th1 cells were expanded and CCR4+ Th2 lymphocytes were reduced, suggesting that abnormal skewing of memory T cell subsets might contribute to reduced antibody synthesis. Despite an expanded number of CD3+CD8+ lymphocytes, increased EBV viral loads occurred in both patients, without overt clinical symptoms of mononucleosis, lymphoproliferative disease or lymphoma. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: Q. Chen, J. L. Cannons, J. C. Paton, H. Akiba, P. L. Schwartzberg, and C. M. Snapper A Novel ICOS-Independent, but CD28- and SAP-Dependent, Pathway of T Cell-Dependent, Polysaccharide-Specific Humoral Immunity in Response to Intact Streptococcus pneumoniae versus Pneumococcal Conjugate Vaccine J. Immunol., December 15, 2008; 181(12): 8258 - 8266. [Abstract] [Full Text] [PDF] I.-J. Kim, C. E. Burkum, T. Cookenham, P. L. Schwartzberg, D. L. Woodland, and M. A. Blackman Perturbation of B Cell Activation in SLAM-Associated Protein-Deficient Mice Is Associated with Changes in Gammaherpesvirus Latency Reservoirs J. Immunol., February 1, 2007; 178(3): 1692 - 1701. [Abstract] [Full Text] [PDF] M. M. McCausland, I. Yusuf, H. Tran, N. Ono, Y. Yanagi, and S. Crotty SAP Regulation of Follicular Helper CD4 T Cell Development and Humoral Immunity Is Independent of SLAM and Fyn Kinase J. Immunol., January 15, 2007; 178(2): 817 - 828. [Abstract] [Full Text] [PDF] S. Crotty, M. M. McCausland, R. D. Aubert, E. J. Wherry, and R. Ahmed Hypogammaglobulinemia and exacerbated CD8 T-cell-mediated immunopathology in SAP-deficient mice with chronic LCMV infection mimics human XLP disease Blood, November 1, 2006; 108(9): 3085 - 3093. [Abstract] [Full Text] [PDF] J. L. Cannons, L. J. Yu, D. Jankovic, S. Crotty, R. Horai, M. Kirby, S. Anderson, A. W. Cheever, A. Sher, and P. L. Schwartzberg SAP regulates T cell-mediated help for humoral immunity by a mechanism distinct from cytokine regulation J. Exp. Med., June 12, 2006; 203(6): 1551 - 1565. [Abstract] [Full Text] [PDF] D. B. Graham, M. P. Bell, M. M. McCausland, C. J. Huntoon, J. van Deursen, W. A. Faubion, S. Crotty, and D. J. McKean Ly9 (CD229)-Deficient Mice Exhibit T Cell Defects yet Do Not Share Several Phenotypic Characteristics Associated with SLAM- and SAP-Deficient Mice J. Immunol., January 1, 2006; 176(1): 291 - 300. [Abstract] [Full Text] [PDF] C. R. Weiler and J. L. Bankers-Fulbright Common Variable Immunodeficiency: Test Indications and Interpretations Mayo Clin. Proc., September 1, 2005; 80(9): 1187 - 1200. [Abstract] [PDF] M. C. Milone, D. E. Tsai, R. L. Hodinka, L. B. Silverman, A. Malbran, M. A. Wasik, and K. E. Nichols Treatment of primary Epstein-Barr virus infection in patients with X-linked lymphoproliferative disease using B-cell-directed therapy Blood, February 1, 2005; 105(3): 994 - 996. [Abstract] [Full Text] [PDF] T. Gross SLAM "dunked"? Blood, May 15, 2004; 103(10): 3613 - 3614. [Full Text] [PDF]

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