Seminars in thrombosis and hemostasis
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Semin. Thromb. Hemost. · Apr 2017
ReviewMonitoring Therapy during Treatment of von Willebrand Disease.
von Willebrand factor (VWF) is an adhesive plasma protein that primarily acts to bridge platelets to sites of vascular injury and thus prevent bleeding. von Willebrand disease (VWD) is the most common inherited bleeding disorder and is caused by deficiency and/or defects of VWF, leading to low levels of plasma VWF or dysfunctional VWF. Factor VIII (FVIII) is also reduced in many patients with VWD, since VWF stabilizes and protects FVIII from degradation. Treatment of VWD primarily entails replacement of VWF, and sometimes FVIII, to protect against bleeding. ⋯ Optimal treatment of VWD entails measuring the effects of treatment, either as a trial before surgery or during therapeutic management. This is usually accomplished by performance of the same tests that are used to help diagnose VWD, although additional monitoring (clinically and/or by laboratory testing) may also be performed. The current review provides an overview of the treatment of VWD but is primarily focused on the monitoring of such therapy.
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Semin. Thromb. Hemost. · Apr 2017
ReviewManaging the Precarious Hemostatic Balance during Extracorporeal Life Support: Implications for Coagulation Laboratories.
For the past four decades, extracorporeal life support (ECLS) has been used to treat critically ill adult and pediatric patients with cardiac and/or respiratory failure unresponsive to medical management, and there are increasing numbers of centers performing ECLS for numerous indications worldwide. Despite the progress with advancing technology, hemorrhagic and thrombotic complications occur frequently and are associated with worse outcomes, but the exact cause is often elusive or multifactorial. ⋯ From an international survey, centers often use a combination of tests to guide management, which in turn can lead to discordant results and confused management. Studies are urgently needed to investigate optimization of current anticoagulation strategies with UFH, as well as use of alternative anticoagulants and nonthrombogenic biomaterials.