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术后血栓性血小板减少性紫癜:综述

Post-operative thrombotic thrombocytopenic purpura: a review.

作者信息

Naqvi T A, Baumann M A, Chang J C

机构信息

Division of Hematology/Oncology, Department of Medicine, Wright State University School of Medicine, Dayton, OH 45428, USA.

出版信息

Int J Clin Pract. 2004 Feb;58(2):169-72. doi: 10.1111/j.1368-5031.2004.0080.x.

Abstract

Post-operative thrombotic thrombocytopenic purpura (TTP) is a recently recognised life-threatening clinical syndrome with considerable similarity to classic TTP in presentation and response to early treatment with plasma exchange. To date, 29 cases of TTP associated with surgery have been reported. The majority of cases have complicated vascular surgeries, with a few cases seen following gastrointestinal or orthopaedic procedures. Characteristically, patients develop microangiopathic haemolytic anaemia and consumptive thrombocytopenia 5 to 9 days following surgery with variable presence of fever, impaired renal function and altered mental status. The pathogenesis of post-operative TTP is speculative but may involve the release of large amounts of high-molecular-weight von Willebrand factor (vWF) multimers due to endothelial damage resulting from surgery in the setting of marginal levels of vWF-cleaving enzyme. The myriad of common post-surgical complications that may present with clinical manifestations similar to TTP may result in confusion with the potential for delay in the initiation of life-saving plasma-exchange therapy. It is important that physicians be alert to the phenomenon of post-operative TTP so that prompt recognition and treatment will prevent serious morbidity or mortality.

摘要

术后血栓性血小板减少性紫癜(TTP)是一种最近才被认识到的危及生命的临床综合征,在临床表现和对血浆置换早期治疗的反应方面与经典TTP有相当大的相似性。迄今为止,已报告29例与手术相关的TTP病例。大多数病例并发于血管手术,少数病例见于胃肠道或骨科手术后。典型的情况是,患者在手术后5至9天出现微血管病性溶血性贫血和消耗性血小板减少,伴有发热、肾功能损害和精神状态改变等不同表现。术后TTP的发病机制尚属推测,但可能涉及在血管性血友病因子裂解酶水平处于临界状态的情况下,手术导致内皮损伤,从而释放大量高分子量血管性血友病因子(vWF)多聚体。众多可能出现与TTP相似临床表现的常见术后并发症,可能会导致混淆,并有可能延误挽救生命的血浆置换治疗的启动。医生必须警惕术后TTP现象,以便及时识别和治疗,防止严重的发病或死亡。

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