SEARCH:   Advanced

Blood, 15 March 2005, Vol. 105, No. 6, pp. 2443-2448. Prepublished online as a Blood First Edition Paper on November 12, 2004; DOI 10.1182/blood-2004-09-3542. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-09-3542v1 105/6/2443    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 Teachey, D. T. Articles by Grupp, S. A. Search for Related Content PubMed PubMed Citation Articles by Teachey, D. T. Articles by Grupp, S. A. Related Collections Hemostasis, Thrombosis, and Vascular Biology Immunobiology Red Cells Apoptosis Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  IMMUNOBIOLOGY Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS) David T. Teachey, Catherine S. Manno, Kelly M. Axsom, Timothy Andrews, John K. Choi, Barbara H. Greenbaum, Joseph M. McMann, Kathleen E. Sullivan, Susan F. Travis, and Stephan A. Grupp From the Division of Oncology, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia; Division of Hematology, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia; Division of Immunology, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia; and the Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia. Autoimmune lymphoproliferative syndrome (ALPS) is a rare disorder of disrupted lymphocyte homeostasis. Clinical manifestations of ALPS vary but typically include autoimmune cytopenias, organomegaly, lymphadenopathy, and increased risk of malignancies. A similar spectrum of symptoms may be seen in some patients with Evans syndrome (ES), a hematologic disorder defined by autoimmune destruction of at least 2 hematologic cell types. We hypothesized that a subset of patients diagnosed with ES may have ALPS. We screened 12 children with ES by flow cytometric analysis for CD4-/CD8- (double negative) T cells (DNTs) and with the definitive test for ALPS, defective in vitro Fas-mediated apoptosis. Six of the patients had elevated DNTs, suggestive of ALPS and also had defective Fas-mediated apoptosis. The other 6 patients displayed normal T-cell apoptosis; 5 of whom had normal DNTs, and 1 had a borderline result. Thus, 7 (58%) of 12 patients with ES had elevated DNTs suggestive of ALPS, with functional confirmation in 6 of 7. This suggests that analysis of DNTs may be a sensitive first-line screening test, serving as a marker of patients who should undergo definitive testing for ALPS. Our data further suggest that a number of patients with ES may have ALPS, a novel finding with important therapeutic implications. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Michel, V. Chanet, A. Dechartres, A.-S. Morin, J.-C. Piette, L. Cirasino, G. Emilia, F. Zaja, M. Ruggeri, E. Andres, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases Blood, October 8, 2009; 114(15): 3167 - 3172. [Abstract] [Full Text] [PDF] D. B. Cines, J. B. Bussel, H. A. Liebman, and E. T. Luning Prak The ITP syndrome: pathogenic and clinical diversity Blood, June 25, 2009; 113(26): 6511 - 6521. [Abstract] [Full Text] [PDF] D. T. Teachey, A. E. Seif, V. I. Brown, M. Bruno, R. M. Bunte, Y. J. Chang, J. K. Choi, J. D. Fish, J. Hall, G. S. Reid, et al. Targeting Notch signaling in autoimmune and lymphoproliferative disease Blood, January 15, 2008; 111(2): 705 - 714. [Abstract] [Full Text] [PDF]

	Copyright © 2005 by American Society of Hematology         Online ISSN: 1528-0020