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真性红细胞增多症患者血小板糖蛋白Ib受体数量增加、血小板黏附增强与严重脑缺血

Increased platelet glycoprotein Ib receptor number, enhanced platelet adhesion and severe cerebral ischaemia in a patient with polycythaemia vera.

作者信息

Toth Judit, Kappelmayer Janos, Udvardy Miklos L, Szanto Timea, Szarvas Mariann, Rejto Laszlo, Soltesz Pal, Udvardy Miklos, Harsfalvi Jolan

机构信息

Clinical Research Center, University of Debrecen, Debrecen, Hungary.

出版信息

Platelets. 2009 Jun;20(4):282-7. doi: 10.1080/09537100902878421.

DOI:10.1080/09537100902878421
PMID:19459135
Abstract

The present study describes severe multiplex cerebral ischaemic laesions in a male patient being diagnosed with polycythaemia vera (PV). In contrast to previous publications, unique platelet receptor pattern with normal platelet count was identified. Glycoprotein Ib receptor number on the surface of resting platelets was increased two-fold and almost three-fold in case of activated platelets compared to the controls. More over, in an in vitro study when whole blood was circulated both at venous and arterial shear conditions and shear rate was adjusted according to the blood viscosity, platelet aggregate/thrombus formation was characteristic on surfaces covered with purified von Willebrand factor while in case of controls the surface was covered with single platelets or platelet monolayer. Similar results with pathological findings have not been published in PV until now. Our result undersigns the necessity of antiplatelet therapy of PV patients, even at normal platelet count.

摘要

本研究描述了一名被诊断为真性红细胞增多症(PV)的男性患者出现的严重多发性脑缺血性病变。与先前的出版物不同,该患者血小板计数正常,但具有独特的血小板受体模式。与对照组相比,静息血小板表面的糖蛋白Ib受体数量增加了两倍,而活化血小板的该受体数量几乎增加了三倍。此外,在一项体外研究中,当全血在静脉和动脉剪切条件下循环并根据血液粘度调整剪切速率时,在覆盖有纯化血管性血友病因子的表面上,血小板聚集/血栓形成具有特征性,而对照组的表面则覆盖着单个血小板或血小板单层。到目前为止,PV患者中尚未发表过与病理结果相似的结果。我们的结果表明,即使血小板计数正常,PV患者也有进行抗血小板治疗的必要性。

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