纤维蛋白原和纤维蛋白溶解紊乱。
Disorders of Fibrinogen and Fibrinolysis.
机构信息
Division of Hematology/Oncology, University of Alabama at Birmingham, 1720 2nd Avenue South, NP 2503, Birmingham, AL 35294, USA.
UNC Department of Pathology and Laboratory Medicine, UNC Blood Research Center, 8018A Mary Ellen Jones Building, CB7035, Chapel Hill, NC 27599-7035, USA.
出版信息
Hematol Oncol Clin North Am. 2021 Dec;35(6):1197-1217. doi: 10.1016/j.hoc.2021.07.011. Epub 2021 Aug 14.
Abstract
Fibrinogen plays a fundamental role in coagulation through its support for platelet aggregation and its conversion to fibrin. Fibrin stabilizes clots and serves as a scaffold and immune effector before being broken down by the fibrinolytic system. Given its importance, abnormalities in fibrin(ogen) and fibrinolysis result in a variety of disorders with hemorrhagic and thrombotic manifestations. This review summarizes (i) the basic elements of fibrin(ogen) and its role in coagulation and the fibrinolytic system; (ii) the laboratory evaluation for fibrin(ogen) disorders, including the use of global fibrinolysis assays; and (iii) the management of congenital and acquired disorders of fibrinogen and fibrinolysis.
摘要
纤维蛋白原在凝血中起着至关重要的作用，它支持血小板聚集并转化为纤维蛋白。纤维蛋白稳定血栓，并在被纤维蛋白溶解系统分解之前充当支架和免疫效应物。鉴于其重要性，纤维蛋白原和纤维蛋白溶解的异常会导致各种具有出血和血栓表现的疾病。这篇综述总结了：（一）纤维蛋白原的基本组成及其在凝血和纤维蛋白溶解系统中的作用；（二）纤维蛋白原疾病的实验室评估，包括使用全球纤维蛋白溶解检测；以及（三）先天性和获得性纤维蛋白原和纤维蛋白溶解异常的治疗。
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