Awada Hassan, Voso Maria Teresa, Guglielmelli Paola, Gurnari Carmelo
Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH 44106, USA.
Department of Biomedicine and Prevention, University of Rome Tor Vergata, 00133 Rome, Italy.
Cancers (Basel). 2020 Jun 30;12(7):1746. doi: 10.3390/cancers12071746.
Over the past decade, new insights have emerged on the pathophysiology of essential thrombocythemia (ET), its clinical management, and associated thrombohemostatic disturbances. Here, we review the latest diagnostic and risk stratification modalities of ET and its therapeutics. Moreover, we discuss the clinical evidence-based benefits, deriving from major clinical trials, of using cytoreductive therapy and antiplatelet agents to lower the risk of fatal vascular events. Also, we focus on the condition of extreme thrombocytosis (>1000 × 10/L) and bleeding risk, the development and pathogenesis of acquired von Willebrand syndrome, and the clinical approach to this paradoxical scenario in ET.
在过去十年中，关于原发性血小板增多症（ET）的病理生理学、临床管理以及相关血栓止血紊乱方面出现了新的见解。在此，我们回顾ET的最新诊断和风险分层方式及其治疗方法。此外，我们讨论了基于主要临床试验证据的、使用细胞减灭疗法和抗血小板药物降低致命性血管事件风险所带来的临床益处。同时，我们关注极端血小板增多症（>1000×10⁹/L）的情况和出血风险、获得性血管性血友病综合征的发展和发病机制，以及ET中这种矛盾情况的临床处理方法。
