Making Sense of the Cytokine Storm: A Conceptual Framework for Understanding, Diagnosing, and Treating Hemophagocytic Syndromes

被引:121
作者
Canna, Scott W. [1 ]
Behrens, Edward M. [1 ]
机构
[1] Childrens Hosp Philadelphia, Div Rheumatol, Philadelphia, PA 19104 USA
关键词
Cytokine storm; Macrophage activation syndrome; Hemophagocytic lymphohistiocytosis; Sepsis; MACROPHAGE ACTIVATION SYNDROME; JUVENILE IDIOPATHIC ARTHRITIS; KILLER-CELL DYSFUNCTION; RHEUMATOID-ARTHRITIS; LYMPHOHISTIOCYTOSIS; MODEL; GENE; DISRUPTION; GUIDELINES; PERFORIN;
D O I
10.1016/j.pcl.2012.03.002
中图分类号
R72 [儿科学];
学科分类号
100202 [儿科学];
摘要
Cytokine storm syndromes (CSS) are a group of disorders representing a variety of inflammatory causes. The clinical presentations of all CSS can be strikingly similar, creating diagnostic uncertainty. However, clinicians should avoid the temptation to treat all CSS equally, because their inciting inflammatory insults vary widely. Failure to identify and address this underlying trigger results in delayed, inoptimal, or potentially harmful consequences. This review places the hemophagocytic syndromes hemophagocytic lymphohistiocytosis and macrophage activation syndrome within a conceptual model of CSS and provides a logical framework for diagnosis and treatment of CSS of suspected rheumatic origin.
引用
收藏
页码:329 / +
页数:17
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