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血栓性微血管病(血栓性血小板减少性紫癜、溶血尿毒综合征、伴有溶血、肝酶升高和血小板减少的子痫前期)

Thrombotic Microangiopathies (TTP, HUS, HELLP).

作者信息

Kappler Shane, Ronan-Bentle Sarah, Graham Autumn

机构信息

Department of Emergency Medicine, Medstar Georgetown University Hospital, Washington Hospital Center, 3800 Reservoir Road, Northwest, Washington, DC 20007, USA.

Department of Emergency Medicine, University of Cincinnati, University Hospital, 231 Albert Sabin Way, PO Box 670769, Cincinnati, OH 45267, USA.

出版信息

Hematol Oncol Clin North Am. 2017 Dec;31(6):1081-1103. doi: 10.1016/j.hoc.2017.08.010.

Abstract

Thrombocytopenia, strictly defined as a platelet count less than 150,000, is common in the emergency department. Recognition, diagnostic investigation, and proper disposition of a thrombocytopenic patient are imperative. One group of disorders leading to thrombocytopenia is the thrombotic microangiopathies, hallmarked by platelet destruction. These thrombotic microangiopathies include thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS) and hemolysis, elevated liver enzyme levels, low platelet count (HELLP), which should be distinguished from similar disease processes such as immune thrombocytopenia (ITP), disseminated intravascular coagulation (DIC) and heparin induced thrombocytopenia (HIT). In this article, clinical presentations, pathophysiology, diagnostic workup, management plans, complications, and dispositions are addressed for this complex group of platelet disorders.

摘要

血小板减少症,严格定义为血小板计数低于150,000,在急诊科很常见。识别、诊断性检查以及对血小板减少症患者的妥善处置至关重要。导致血小板减少症的一组疾病是血栓性微血管病,其特征是血小板破坏。这些血栓性微血管病包括血栓性血小板减少性紫癜(TTP)、溶血性尿毒症综合征(HUS)以及溶血、肝酶水平升高、血小板计数降低(HELLP),应将其与免疫性血小板减少症(ITP)、弥散性血管内凝血(DIC)和肝素诱导的血小板减少症(HIT)等类似疾病过程区分开来。本文讨论了这一复杂的血小板疾病组的临床表现、病理生理学、诊断检查、管理计划、并发症及处置。

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