Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
Blood. 2005 Feb 15;105(4):1648-51. Epub 2004 Oct 5.

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.

Author information

  • 1Pediatric Rheumatology, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium. an.billiau@med.kuleuven.ac.be

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.

PMID:
15466922
[PubMed - indexed for MEDLINE]
Free full text
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Icon for HighWire
    Loading ...
    Write to the Help Desk