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Blood, 15 February 2005, Vol. 105, No. 4, pp. 1648-1651. Prepublished online as a Blood First Edition Paper on October 5, 2004; DOI 10.1182/blood-2004-08-2997. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-08-2997v1 105/4/1648    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 Billiau, A. D. Articles by Wouters, C. Search for Related Content PubMed PubMed Citation Articles by Billiau, A. D. Articles by Wouters, C. Related Collections Immunobiology Phagocytes Free Research Articles Brief Reports Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  IMMUNOBIOLOGY Brief report Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN--producing lymphocytes and IL-6- and TNF--producing macrophages An D. Billiau, Tania Roskams, Rita Van Damme-Lombaerts, Patrick Matthys, and Carine Wouters From the Pediatric Rheumatology, University Hospital Gasthuisberg, Leuven, Belgium; Laboratory of Experimental Transplantation, University of Leuven, Belgium; Department of Histopathology, University Hospital Gasthuisberg, Leuven, Belgium; Pediatric Transplantation, University Hospital Gasthuisberg, Leuven, Belgium; and Laboratory of Immunobiology, Rega-Institute for Medical Research, University of Leuven, Belgium. Macrophage activation syndrome (MAS) is a rare and potentially fatal disorder, thought to result from uncontrolled activation and proliferation of T cells and excessive activation of macrophages. The term MAS designates a clinicopathologic entity that occurs in different hemophagocytic syndromes (HSs). Primary hemophagocytic lymphohistiocytosis (HLH) is recognized to have an immunogenetic basis, but in the secondary HS (also referred to as secondary HLH), the cause is unknown. The pathogenesis of the accelerated disease phase typical of MAS remains incompletely understood. This report describes the immunohistochemical findings on liver tissues from 5 children, each of whom presented with MAS in the context of a different type of HS. The data provide direct evidence for the involvement of activated CD8+ lymphocytes through the production of interferon- and of macrophages through hemophagocytosis and production of interleukin 6 and tumor necrosis factor-, and underscore the view that MAS in different HSs share a common effector pathway. (Blood. 2005;105:1648-1651) CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: A series of unfortunate events Michael B. Jordan Blood 2005 105: 1372-1373. [Full Text] [PDF] This article has been cited by other articles: S Ishikawa, T Mima, C Aoki, N Yoshio-Hoshino, Y Adachi, T Imagawa, M Mori, M Tomiita, N Iwata, T Murata, et al. Abnormal expression of the genes involved in cytokine networks and mitochondrial function in systemic juvenile idiopathic arthritis identified by DNA microarray analysis Ann Rheum Dis, February 1, 2009; 68(2): 264 - 272. [Abstract] [Full Text] [PDF] N. Mahlaoui, M. Ouachee-Chardin, G. de Saint Basile, B. Neven, C. Picard, S. Blanche, and A. Fischer Immunotherapy of Familial Hemophagocytic Lymphohistiocytosis With Antithymocyte Globulins: A Single-Center Retrospective Report of 38 Patients Pediatrics, September 1, 2007; 120(3): e622 - e628. [Abstract] [Full Text] [PDF] M. Ouachee-Chardin, C. Elie, G. de Saint Basile, F. Le Deist, N. Mahlaoui, C. Picard, B. Neven, J.-L. Casanova, M. Tardieu, M. Cavazzana-Calvo, et al. Hematopoietic Stem Cell Transplantation in Hemophagocytic Lymphohistiocytosis: A Single-Center Report of 48 Patients Pediatrics, April 1, 2006; 117(4): e743 - e750. [Abstract] [Full Text] [PDF]

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