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eISSN: 2084-9834
ISSN: 0034-6233
Reumatologia/Rheumatology
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6/2013
vol. 51
 
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abstract:
Review paper

Macrophage activation syndrome

Bożena Rojek-Margas
,
Beata Śliwowska
,
Jolanta Bucka

Reumatologia 2013; 51, 6: 459–466
Online publish date: 2013/12/24
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Macrophage activation syndrome (MAS) is a severe complication of systemic connective tissue diseases, particularly systemic juvenile idiopathic arthritis, systemic lupus erythematosus and adult-onset Still’s disease. It is a life-threatening disorder arising from immunoregulation inherited or acquired dysfunction of the NK cells and cytotoxic T-lymphocytes. It is characterized by multi-organ failure with a number of clinical symptoms. So far, there is no standard procedure for treatment. Glucocorticoids, intravenous immunoglobulin, sometimes cyclosporine A and etoposide are used.
keywords:

macrophage activation syndrome, hemophagocytic syndrome, hemophagocytic lymphohistiocytosis







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