Title: Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-gamma-producing lymphocytes and IL-6- and TNF-alpha-producing macrophages
Authors: Billiau, An
Roskams, Tania
Lombaerts, Rita
Matthys, Patrick
Wouters, Carine # ×
Issue Date: Feb-2005
Series Title: Blood vol:105 issue:4 pages:1648-1651
Abstract: 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-gamma and of macrophages through hemophagocytosis and production of interleukin 6 and tumor necrosis factor-alpha, and underscore the view that MAS in different HSs share a common effector pathway.
ISSN: 0006-4971
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Translational Cell & Tissue Research
Laboratory of Immunobiology (Rega Institute)
Laboratory of Pediatric Immunology
Laboratory of Experimental Transplantation
× corresponding author
# (joint) last author

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