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Parahemophilia:因子 V 缺乏症的新见解。

Parahemophilia: new insights into factor v deficiency.

机构信息

Division of Hematology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

出版信息

Semin Thromb Hemost. 2013 Sep;39(6):607-12. doi: 10.1055/s-0033-1349224. Epub 2013 Jul 26.

Abstract

Blood coagulation factor V (FV) plays a pivotal role in blood coagulation. It is found in both plasma and in platelets and has a profound impact on thrombin generation. Deficiency of this clotting factor due to inherited or acquired conditions results in a broad spectrum of bleeding symptoms. Surprisingly however, some patients with undetectable levels of FV experience relatively mild bleeding. The aim of this review is to highlight this rare coagulation factor disorder and touch upon its clinical manifestations, diagnosis, and treatment. Furthermore, recent advances that shed new light on the importance of platelet FV and other modifiers which influence bleeding tendencies in severe FV deficiency will be discussed.

摘要

凝血因子 V(FV)在血液凝固中起着关键作用。它存在于血浆和血小板中,对凝血酶的生成有深远的影响。由于遗传或获得性原因导致这种凝血因子缺乏,会导致广泛的出血症状。然而,令人惊讶的是,一些 FV 水平无法检测到的患者出血症状相对较轻。本文的目的是强调这种罕见的凝血因子紊乱,并探讨其临床表现、诊断和治疗。此外,还将讨论最近的一些进展,这些进展揭示了血小板 FV 的重要性以及其他影响严重 FV 缺乏症出血倾向的修饰因子。

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