SEARCH:   Advanced

This Article Full Text Full Text (PDF) 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 Emile, J.-F. Articles by Fischer, A. Search for Related Content PubMed PubMed Citation Articles by Emile, J.-F. Articles by Fischer, A. Related Collections Immunobiology Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, Vol. 96 No. 1 (July 1), 2000: pp. 58-62 Langerhans cell deficiency in reticular dysgenesis Jean-François Emile, Frédéric Geissmann, Oscar de la Calle Martin, Isabelle Radford-Weiss, Yves Lepelletier, Berno Heymer, Teresa Espanol, Ken B. de Santes, Yves Bertrand, Nicole Brousse, Jean-Laurent Casanova, and Alain Fischer From the Service d'Anatomie Pathologique, Hôpital Paul Brousse, and UPRES 1596, Université Paris-Sud, Villejuif; UMR 8603 CNRS/Université Paris V, Service d'Histo-Embryologie, Hôpital, Service d'Anatomie Pathologique, Département de Pédiatrie and INSERM U429, Hôpital Necker-Enfants Malades, Paris; Département de Pédiatrie, Hôpital Debrousse, Lyon, France; the Department of Immunology, Hospital de la Santa Creu i Sant Paul, and the Immunology Unit, C. S. Vall d'Hebron, Barcelona, Spain; Abteilung für Pathologie, Universität Ulm, Ulm, Germany; and the Department of Pediatrics, University Hospital and Clinics, Madison, WI. Reticular dysgenesis is a rare inherited immunodeficiency characterized by the lack of blood monocytes and neutrophils and low lymphocyte counts, contrasting with normal red blood cell counts and normal or decreased platelet counts. Whether dendritic cells or macrophages, both of which derive primarily from blood monocytes, are affected in this condition remains unknown. We studied 7 patients with reticular dysgenesis. Macrophages were present in normal numbers in the dermis and in the atrophic lymphoid tissues of these patients, proving that at least some subsets of macrophages can differentiate despite very low monocyte counts. By contrast, Langerhans cells, which are CD1a-positive epidermal dendritic cells, were absent in all (n = 5) patients before bone marrow transplantation. After bone marrow transplantation, Langerhans cells were present (n = 2), suggesting that the defect is not related to keratinocyte dysfunction. A split chimeric reconstitution, characterized by the presence of autologous blood monocytes able to differentiate in vitro into CD1a-positive dendritic cells, was observed in a patient who underwent successful engraftment. These results suggest that an intrinsic cell defect is unlikely and that a bone marrow-derived factor may be defective in reticular dysgenesis; it may be responsible for the Langerhans cell defect but not involved in macrophage differentiation. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. Maheshwari and R. D. Christensen Neutropenia in the Neonatal Intensive Care Unit NeoReviews, October 1, 2004; 5(10): e431 - e443. [Full Text] [PDF]

	Copyright © 2000 by American Society of Hematology         Online ISSN: 1528-0020