1. Novel antiplatelet strategies targeting VWF and GPIb
- Author
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Marie Scully and Nithya Prasannan
- Subjects
0301 basic medicine ,Thrombotic microangiopathy ,Thrombotic thrombocytopenic purpura ,Disease ,030204 cardiovascular system & hematology ,Bioinformatics ,03 medical and health sciences ,0302 clinical medicine ,Von Willebrand factor ,hemic and lymphatic diseases ,von Willebrand Factor ,medicine ,Humans ,Platelet ,biology ,business.industry ,Autoantibody ,Hematology ,General Medicine ,medicine.disease ,ADAMTS13 ,030104 developmental biology ,Platelet Glycoprotein GPIb-IX Complex ,biology.protein ,Caplacizumab ,business ,Platelet Aggregation Inhibitors - Abstract
Von Willebrand factor has a pivotal role in primary hemostasis. Its role in thrombotic microangiopathies (TMA), as well as cardiovascular disease, has been demonstrated. Thrombotic thrombocytopenic purpura (TTP), a thrombotic microangiopathy, is a life-threatening condition with a high mortality rate if untreated. Current management strategies comprise plasma exchange to remove autoantibodies and replenish ADAMTS13, along with immunosuppressive agents in immune TTP. This review focuses on novel antiplatelet strategies that target VWF and GPIb. The benefits of the nanobody caplacizumab in achieving faster normalization of platelet count, as well as reduced thromboembolic events were shown through TITAN and HERCULES trials, and these findings have been practice changing. The use of caplacizumab in patients with immune TTP (iTTP) has now become well established. Potential benefits of ARC1779 and N-acetylcysteine have also been shown on a small scale in iTTP, however these lack evidence through larger randomized controlled trials. Further therapies, some in early phase, others in clinical practice, target platelet aggregation within arteries and their utility is presented with cerebrovascular disorders.
- Published
- 2020
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