Making sense of the cytokine storm: a conceptual framework for understanding, diagnosing, and treating hemophagocytic syndromes.

Making sense of the cytokine storm: a conceptual framework for understanding, diagnosing, and treating hemophagocytic syndromes.
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理解细胞因子风暴:理解,诊断和治疗造血综合征的概念框架。

DOI:
10.1016/j.pcl.2012.03.002
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发表时间:
2012-04
影响因子:
2.6
通讯作者:
Behrens, Edward M.
Behrens, Edward M.
中科院分区:
医学3区
文献类型:
--
作者:
Canna, Scott W.;Behrens, Edward M.

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细胞因子风暴综合征(CSS)是一组代表各种炎症病因的疾病,最终常见结果是压倒性全身炎症、血流动力学不稳定、多器官功能障碍和潜在死亡。噬血细胞综合征噬血细胞性淋巴组织细胞增多症(HLH)和巨噬细胞活化综合征(MAS)代表两种临床相似的CSS,但病理学重叠程度未知。所有CSS的临床表现可能惊人地相似,造成诊断的不确定性。然而,临床医生应该避免平等对待所有CSS的诱惑,因为它们的煽动性炎症损伤差异很大。未能识别和解决这一潜在的触发因素将导致延迟、不理想或潜在的有害后果。本文综述了噬血细胞综合征HLH和MAS的CSS的概念模型,从而提供了一个逻辑框架的诊断和治疗CSS的疑似风湿性起源。
Cytokine Storm Syndromes (CSS) are a group of disorders representing a variety of inflammatory etiologies with the final common result of overwhelming systemic inflammation, hemodynamic instability, multiple organ dysfunction, and potentially death. The hemophagocytic syndromes hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) represent two clinically similar CSS with an unknown degree of pathoetiologic overlap. The clinical presentations of all CSS can be strikingly similar, creating diagnostic uncertainty. However, clinicians should avoid the temptation to treat all CSS equally, as their inciting inflammatory insults vary widely. Failure to identify and address this underlying trigger will result in delayed, inoptimal, or potentially harmful consequences. This review endeavors to place the hemophagocytic syndromes HLH and MAS within a conceptual model of CSS, and thus provide a logical framework for diagnosis and treatment of CSS of suspected rheumatic origin.
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