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血小板黏附综合征:危及生命的血栓形成并发症的一个重要原因。

Sticky platelet syndrome: an important cause of life-threatening thrombotic complications.

作者信息

Kubisz Peter, Stanciakova Lucia, Stasko Jan, Dobrotova Miroslava, Skerenova Maria, Ivankova Jela, Holly Pavol

机构信息

a National Center of Hemostasis and Thrombosis, Clinic of Hematology and Transfusiology, Jessenius Faculty of Medicine in Martin , Comenius University in Bratislava, Martin University Hospital , Martin , Slovak Republic.

b Department of Clinical Biochemistry, Jessenius Faculty of Medicine in Martin , Comenius University in Bratislava, Martin University Hospital , Martin , Slovak Republic.

出版信息

Expert Rev Hematol. 2016 Jan;9(1):21-35. doi: 10.1586/17474086.2016.1121095. Epub 2015 Dec 9.

DOI:10.1586/17474086.2016.1121095
PMID:26567442
Abstract

Sticky platelet syndrome (SPS) is a prothrombotic thrombocytopathy with familial occurrence, characterized by hyperaggregability of platelets in response to adenosine diphosphate (ADP), epinephrine (EPI) or both. The syndrome has been identified in approximately 21% of unexplained arterial thrombotic episodes, regarded to be the most common thrombophilia in arterial thrombosis and 13.2% of unexplained venous thromboembolism (VTE). The relatively young age at the first manifestation, relation to fertility and pregnancy, seriousness of the symptoms, easy and effective management of the disorder indicate to the necessity to take it into account in the differential diagnosis of the underlying cause of the thrombotic event. As the various localizations of the thrombosis in SPS have been reported, its management often requires a multidisciplinary approach. This review deals with the clinical aspects of thrombophilia, its etiopathogenesis, diagnosis as well as novel advances in the treatment and outlines the challenges for the further research.

摘要

黏附性血小板综合征(SPS)是一种具有家族性的血栓前血小板病,其特征是血小板对二磷酸腺苷(ADP)、肾上腺素(EPI)或两者产生过度聚集反应。该综合征在约21%无法解释的动脉血栓形成事件中被发现,被认为是动脉血栓形成中最常见的易栓症,在13.2%无法解释的静脉血栓栓塞(VTE)中也有发现。首次发病时相对年轻的年龄、与生育和妊娠的关系、症状的严重性、该疾病易于且有效的管理表明在鉴别诊断血栓形成事件的潜在原因时必须考虑到它。由于SPS中血栓形成的各种部位已有报道,其管理通常需要多学科方法。本综述探讨了易栓症的临床方面、其病因发病机制、诊断以及治疗方面的新进展,并概述了进一步研究面临的挑战。

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