Document Detail

Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities?
MedLine Citation:
PMID:  12960485     Owner:  NLM     Status:  MEDLINE    
PURPOSE OF THE REVIEW: One of the most perplexing features of systemic-onset juvenile rheumatoid arthritis is the association with macrophage activation syndrome, a life-threatening complication caused by excessive activation and proliferation of T cells and macrophages. The main purpose of the review is to summarize current understanding of the relation between macrophage activation syndrome and other clinically similar hemophagocytic disorders.
RECENT FINDINGS: Clinically, macrophage activation syndrome has strong similarities with familial and virus-associated reactive hemophagocytic lymphohistiocytosis. The better understood familial hemophagocytic lymphohistiocytosis is a constellation of rare, autosomal recessive immune disorders. The most consistent immunologic abnormalities in patients with familial hemophagocytic lymphohistiocytosis are decreased natural killer and cytotoxic cell functions. In approximately one third of familial hemophagocytic lymphohistiocytosis patients, these immunologic abnormalities are secondary to mutations in the gene encoding perforin, a protein that mediates cytotoxic activity of natural killer and cytotoxic CD8+ T cells. Several recent studies have suggested that profoundly depressed natural killer cell activity and abnormal levels of perforin expression may be a feature of macrophage activation syndrome in systemic-onset juvenile rheumatoid arthritis as well. Although it has been proposed that in both hemophagocytic lymphohistiocytosis and macrophage activation syndrome, natural killer and cytotoxic cell dysfunction may lead to inadequate control of cellular immune responses, the exact nature of such dysregulation and the relation between macrophage activation syndrome and hemophagocytic lymphohistiocytosis still remain to be determined.
Alexei A Grom
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Current opinion in rheumatology     Volume:  15     ISSN:  1040-8711     ISO Abbreviation:  Curr Opin Rheumatol     Publication Date:  2003 Sep 
Date Detail:
Created Date:  2003-09-08     Completed Date:  2003-12-16     Revised Date:  2013-05-20    
Medline Journal Info:
Nlm Unique ID:  9000851     Medline TA:  Curr Opin Rheumatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  587-90     Citation Subset:  IM    
Cincinnati Children's Hospital Medical Center, OH 45215, USA.
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MeSH Terms
Arthritis, Juvenile Rheumatoid / complications,  pathology,  physiopathology
Histiocytosis, Non-Langerhans-Cell / etiology,  pathology,  physiopathology*
Killer Cells, Natural / physiology
Macrophage Activation*
Terminology as Topic*

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