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黏附性血小板综合征。

Sticky platelet syndrome.

机构信息

Department of Hematology and Transfusion Medicine, Jessenius Faculty of Medicine of the Comenius University, University Hospital, Martin, Slovakia.

出版信息

Semin Thromb Hemost. 2013 Sep;39(6):674-83. doi: 10.1055/s-0033-1353394. Epub 2013 Aug 10.

DOI:10.1055/s-0033-1353394
PMID:23934738
Abstract

Sticky platelet syndrome (SPS) is a thrombophilic thrombocytopathy with familial occurrence and autosomal dominant trait, characterized by an increased in vitro platelet aggregation in response to low concentrations of adenosine diphosphate (ADP) and/or epinephrine (EPI). According to aggregation pattern, three types of the syndrome can be identified (hyperresponse after both reagents, Type I; EPI alone, Type II; ADP alone, Type III). Clinically, the syndrome is associated with both venous and arterial thrombosis. In pregnant women, complications such as fetal growth retardation and fetal loss have been reported. The first thrombotic event usually occurs before 40 years of age and without prominent acquired risk factors. Antiplatelet drugs generally represent adequate treatment. The use of other antithrombotics is usually ineffective and may result in the recurrence of thrombosis. In most patients, low doses of antiplatelet drugs (acetylsalicylic acid, 80-100 mg/d) lead to normalization of hyperaggregability. Combination of SPS with other thrombophilic disorders has been described. Despite several studies investigating platelet glycoproteins' role in platelets' activation and aggregation, the precise defect responsible for the syndrome remains unknown. The aim of this review is to summarize authors' own experience about SPS and the clinical data indexed in selected databases of medical literature (PubMed and Scopus).

摘要

黏附血小板综合征(SPS)是一种具有家族性和常染色体显性遗传特征的血栓形成性血小板增多症,其特征是体外对低浓度二磷酸腺苷(ADP)和/或肾上腺素(EPI)的血小板聚集反应增加。根据聚集模式,可识别出三种类型的综合征(两种试剂后高反应,I 型;仅 EPI,II 型;仅 ADP,III 型)。临床上,该综合征与静脉和动脉血栓形成有关。在孕妇中,已报道了胎儿生长迟缓和胎儿丢失等并发症。首次血栓事件通常发生在 40 岁之前,且无明显的后天危险因素。抗血小板药物通常是有效的治疗方法。其他抗血栓药物的使用通常无效,可能导致血栓再次形成。在大多数患者中,低剂量的抗血小板药物(乙酰水杨酸,80-100mg/d)可使高聚集性正常化。已描述了 SPS 与其他血栓形成性疾病的联合。尽管有几项研究调查了血小板糖蛋白在血小板激活和聚集中的作用,但导致该综合征的确切缺陷仍不清楚。本文综述的目的是总结作者自己关于 SPS 的经验以及在选定的医学文献数据库(PubMed 和 Scopus)中索引的临床数据。

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