Interplay between coagulation and vascular inflammation in sickle cell disease.

Interplay between coagulation and vascular inflammation in sickle cell disease.
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DOI:
10.1111/bjh.12336
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发表时间:
2013-07
影响因子:
6.5
通讯作者:
Pawlinski R
Pawlinski R
中科院分区:
医学2区
文献类型:
--
作者:
Sparkenbaugh E;Pawlinski R

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镰状细胞病是最常见的遗传性血液疾病,可导致多个器官的不可逆损伤。尽管红细胞镰状化和血管闭塞是镰状细胞病病理生理学的核心,但溶血性贫血和血管病变的重要性最近已得到认识。高凝状态是镰状细胞病的另一个显着特征,由内在和外在凝血途径的激活介导。越来越多的证据表明,凝血不仅可能导致血栓并发症,而且可能导致与这种疾病相关的血管炎症。本文总结了血管炎症和凝血激活的作用,讨论了凝血激活的潜在机制,并回顾了证明镰状细胞病中凝血和血管炎症之间相互影响的最新数据。
Sickle cell disease is the most common inherited hematologic disorder that leads to the irreversible damage of multiple organs. Although sickling of red blood cells and vaso-occlusion are central to the pathophysiology of sickle cell disease the importance of hemolytic anemia and vasculopathy has been recently recognized. Hypercoagulation state is another prominent feature of sickle cell disease and is mediated by activation of both intrinsic and extrinsic coagulation pathways. Growing evidence demonstrates that coagulation may not only contribute to the thrombotic complications, but also to vascular inflammation associated with this disease. This article summarizes the role of vascular inflammation and coagulation activation, discusses potential mechanisms responsible for activation of coagulation and reviews recent data demonstrating the crosstalk between coagulation and vascular inflammation in sickle cell disease.
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