Gowert Nina Sarah, Klier Meike, Reich Maria, Reusswig Friedrich, Donner Lili, Keitel Verena, Häussinger Dieter, Elvers Margitta
Department of Clinical and Experimental Hemostasis, Hemotherapy and Transfusion Medicine, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany.
Department of Vascular and Endovascular Surgery, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany.
Cell Physiol Biochem. 2017;41(6):2133-2149. doi: 10.1159/000475566. Epub 2017 Apr 20.
BACKGROUND/AIMS: Platelets are essential mediators of hemostasis to avoid excessive blood loss. Cirrhosis and chronic liver diseases are characterized by alterations in hemostasis. Alterations in the secondary hemostasis have been well studied, while defects in primary hemostasis, especially the consequences of cholestatic liver disease on platelet function are not well defined.
After bile duct ligation (BDL) platelet activation and thrombus formation were analyzed in mice.
BDL in mice had a moderate effect on platelet counts; however, intrinsic platelet activation was strongly reduced upon activation of the collagen receptor GPVI at early time points. 7 days after bile duct ligation, platelets displayed an almost complete loss of activation with reduced agonist-triggered release of alpha and dense granules and expression of integrin αIIbβ3 on the platelet surface. This activation defects resulted in strongly reduced thrombus formation under flow, reduced platelet adhesion to fibrinogen and bleeding complications in BDL mice as measured by tail bleeding experiments. Mechanistically, elevated nitric oxide and prostacyclin levels induced phosphorylation of Vasodilator-stimulated phosphoprotein (VASP), an established inhibitor of platelet activation. Furthermore increased tissue plasminogen activator in plasma of BDL mice led to enhanced plasmin levels that might be responsible for reduced glycoprotein expression of BDL platelets. Besides, high amounts of bile acids contribute to defective signal transduction as shown in platelets from mice fed with a cholic acid diet.
Cholestatic liver disease induces multiple platelet activation defects and impairs thrombus formation responsible for bleeding complications at least in mice.
背景/目的:血小板是止血过程中避免过度失血的重要介质。肝硬化和慢性肝病的特征是止血功能改变。继发性止血的改变已得到充分研究,而原发性止血缺陷,尤其是胆汁淤积性肝病对血小板功能的影响尚不明确。
在小鼠中进行胆管结扎(BDL)后,分析血小板活化和血栓形成情况。
小鼠胆管结扎对血小板计数有中等程度影响;然而,在早期时间点,胶原受体糖蛋白VI(GPVI)激活时,血小板内在活化显著降低。胆管结扎7天后,血小板几乎完全丧失活化能力,α颗粒和致密颗粒的激动剂触发释放减少,血小板表面整合素αIIbβ3表达降低。这种活化缺陷导致血流状态下血栓形成显著减少、血小板对纤维蛋白原的黏附降低,通过尾部出血实验检测发现BDL小鼠出现出血并发症。机制上,一氧化氮和前列环素水平升高诱导血管舒张刺激磷蛋白(VASP)磷酸化,VASP是一种已确定的血小板活化抑制剂。此外,BDL小鼠血浆中组织纤溶酶原激活物增加导致纤溶酶水平升高,这可能是BDL血小板糖蛋白表达降低的原因。此外,如喂食胆酸饮食的小鼠血小板所示,大量胆汁酸导致信号转导缺陷。
胆汁淤积性肝病至少在小鼠中诱导多种血小板活化缺陷并损害血栓形成,导致出血并发症。