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血小板增多症中出血与血栓形成的悖论:血管性血友病因子是其中的联系吗?

The paradox of bleeding and thrombosis in thrombocythemia: is von Willebrand factor the link?

作者信息

van Genderen P J, Leenknegt H, Michiels J J

机构信息

Department of Internal Medicine II and Hematology, Erasmus University Dijkzigt, Rotterdam, The Netherlands.

出版信息

Semin Thromb Hemost. 1997;23(4):385-9. doi: 10.1055/s-2007-996113.

Abstract

Thrombosis and bleeding, reflecting the extreme ends of hemostasis, are common complications in patients with thrombocythemia in its primary form or in association with polycythemia vera. The pathogenesis of these thrombohemorrhagic predispositions is not clearly understood. In the present study we describe the paradoxical and sequential occurrence of thrombotic and bleeding complications in six patients with thrombocythemia. Strikingly, in all patients some abnormality of plasma von Willebrand factor was detected. The potential role of von Willebrand factor in the pathogenesis of these hemostatic complications is discussed.

摘要

血栓形成和出血反映了止血的两个极端情况,是原发性血小板增多症患者或与真性红细胞增多症相关的患者常见的并发症。这些血栓出血倾向的发病机制尚不清楚。在本研究中,我们描述了6例血小板增多症患者血栓形成和出血并发症的矛盾性及相继发生情况。令人惊讶的是,在所有患者中均检测到血浆血管性血友病因子存在某种异常。本文讨论了血管性血友病因子在这些止血并发症发病机制中的潜在作用。

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