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Blood, 6 May 2010, Vol. 115, No. 18, pp. 3708-3717. Prepublished online as a Blood First Edition Paper on December 28, 2009; DOI 10.1182/blood-2009-02-202796. This Article Full Text (PDF) Supplemental Table and Figures All Versions of this Article: blood-2009-02-202796v1 115/18/3708    most recent e-Letter: Submit a Response Alert me when this article is cited Alert me when e-Letters are posted 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 Scott-Algara, D. Articles by Lortholary, O. Search for Related Content PubMed PubMed Citation Articles by Scott-Algara, D. Articles by Lortholary, O. Related Collections Related Article in Blood Online Social Bookmarking                         What's this?  Previous Article  |  Next Article  Submitted February 9, 2009; accepted December 6, 2009. Idiopathic CD4+ T-cell lymphocytopenia is associated with impaired membrane expression of the chemokine receptor CXCR4 Daniel Scott-Algara1, Karl Balabanian2, Lisa A. Chakrabarti3, Luc Mouthon4, Francoise Dromer5, Celine Didier1, Fernando Arenzana-Seisdedos6 and Olivier Lortholary7,* 1 Institut Pasteur, Unite de Regulation des Infections Retrovirales, Paris, France; 2 INSERM U996, Universite Paris-Sud 11, Clamart, France; 3 Institut Pasteur, Unite d'Immunogenetique Cellulaire, Paris, France; 4 Universite Paris Descartes, Pole de Medecine Interne, UPRES EA 4058, Hopital Cochin, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France; 5 Institut Pasteur, Unite de Mycologie Moleculaire and CNRS URA3012, Paris, France; 6 Institut Pasteur, Laboratoire de Pathogenie Virale, Paris, France; 7 Universite Paris Descartes, Service des Maladies Infectieuses et Tropicales, Centre d'Infectiologie Necker-Pasteur, Hopital Necker-Enfants Malades, AP-HP, Paris, France * Corresponding author; email: olortho{at}pasteur.fr Abstract Idiopathic CD4+ T-cell lymphocytopenia (ICL) is a rare acquired T-cell immunodeficiency of unknown pathogenic basis. Six adults with ICL who developed opportunistic infections were investigated using extensive immunophenotyping analysis and functional evaluation of the chemokine receptor CXCR4. For all 6 patients studied, a profound defect in CXCR4 expression was detected at the surface of CD4+ T lymphocytes, in association with an abnormal intracellular accumulation of CXCR4 and of its natural ligand, the chemokine CXCL12. For all patients studied, CD4+ T-cell chemotactic response towards CXCL12 was decreased, while sensitivity to CXCL8 was preserved. CXCR4 recovery following ligand-induced endocytosis was impaired in ICL CD4+ T-cells. Upon in vitro addition of IL-2, membrane expression of CXCR4 returned to normal levels in 5/6 patients, while intracellular accumulation of CXCR4 and CXCL12 disappeared. Upon therapeutic administration of IL-2, CD4+ T-cell count and membrane CXCR4 expression and function improved over time in three of four patients treated. Therefore, our data indicate that ICL is associated with defective surface expression of CXCR4, which may be reversed by IL-2. CiteULike    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this? Related Article in Blood Online: Changing lanes in ICL Irini Sereti Blood 2010 115: 3648-3649. [Full Text] [PDF] This article has been cited by other articles: I. Sereti Changing lanes in ICL Blood, May 6, 2010; 115(18): 3648 - 3649. [Full Text] [PDF]

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