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重症监护中的噬血细胞综合征:伪装者。

Haemophagocytic syndrome in intensive care: The great pretender.

作者信息

de Asua Ignacio, Ciliberti Esteban

机构信息

Department of Anaesthetics and Intensive Care, Royal Brompton and Harefield NHS Trust, Harefield, Middlesex, UK.

出版信息

J Intensive Care Soc. 2015 Nov;16(4):316-319. doi: 10.1177/1751143715584383. Epub 2015 Apr 29.

DOI:10.1177/1751143715584383
PMID:28979438
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5606461/
Abstract

The haemophagocytic syndrome is a unique disease process in which the dysregulation of the patient's immune system leads to an inflammatory storm which rapidly ravages through multiple organ systems, generating life-threatening end-organ dysfunction. Since it usually mimics other conditions frequently encountered in the critical care population - most notably septic shock - its diagnosis is elusive and the condition remains under-recognized and under-reported. We present a concise review of the pathophysiology and clinical features of the heamophagocytic syndrome and discuss the main diagnostic and therapeutic issues relevant to the management of this condition in the critically ill patient. Increasing awareness about the haemophagocytic syndrome amongst intensive care physicians will facilitate earlier recognition and timely management.

摘要

噬血细胞综合征是一种独特的疾病过程,其中患者免疫系统的失调会引发炎症风暴,迅速侵袭多个器官系统,导致危及生命的终末器官功能障碍。由于它通常类似于重症监护人群中常见的其他病症——最显著的是脓毒性休克——其诊断难以捉摸,该病症仍未得到充分认识和报告。我们简要综述了噬血细胞综合征的病理生理学和临床特征,并讨论了与危重症患者该病症管理相关的主要诊断和治疗问题。提高重症监护医生对噬血细胞综合征的认识将有助于早期识别和及时管理。

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本文引用的文献

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Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features, predictive factors, and prognosis in 21 patients.成人斯蒂尔病中的反应性噬血细胞综合征:21例患者的临床特征、预测因素及预后
Medicine (Baltimore). 2015 Jan;94(4):e451. doi: 10.1097/MD.0000000000000451.
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Bone marrow assessment in the diagnosis of acquired hemophagocytic lymphohistiocytosis in adults.成人获得性噬血细胞性淋巴组织细胞增生症诊断中的骨髓评估
Am J Clin Pathol. 2015 Feb;143(2):308-9. doi: 10.1309/AJCPUK8TLI2MLYOQ.
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Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis.噬血细胞性淋巴组织细胞增生症成人患者的临床特征、预后因素和转归。
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Prognostic factors of early death in a cohort of 162 adult haemophagocytic syndrome: impact of triggering disease and early treatment with etoposide.162例成人噬血细胞综合征队列中早期死亡的预后因素:触发疾病及依托泊苷早期治疗的影响
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Apoptotic cell clearance: basic biology and therapeutic potential.细胞凋亡清除:基础生物学与治疗潜能。
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Lancet. 2014 Apr 26;383(9927):1503-1516. doi: 10.1016/S0140-6736(13)61048-X. Epub 2013 Nov 27.
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Advances in understanding the pathogenesis of HLH.理解 HLH 发病机制的研究进展。
Br J Haematol. 2013 Jun;161(5):609-622. doi: 10.1111/bjh.12293. Epub 2013 Apr 12.
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Br J Haematol. 2013 Feb;160(3):275-87. doi: 10.1111/bjh.12138. Epub 2012 Dec 4.
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J Immunol Methods. 2011 Feb 1;364(1-2):1-13. doi: 10.1016/j.jim.2010.11.006. Epub 2010 Nov 24.