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Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding

1
Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH 44106, USA
2
Department of Biomedicine and Prevention, University of Rome Tor Vergata, 00133 Rome, Italy
3
Fondazione Santa Lucia, Laboratorio di Neuro-Oncoematologia, 00143 Roma, Italy
4
CRIMM-Centro Ricerca e Innovazione delle Malattie Mieloproliferative, Department of Experimental and Clinical Medicine, Azienda ospedaliera-Universitaria Careggi, University of Florence, 50139 Florence, Italy
*
Author to whom correspondence should be addressed.
Cancers 2020, 12(7), 1746; https://doi.org/10.3390/cancers12071746
Received: 8 June 2020 / Revised: 28 June 2020 / Accepted: 29 June 2020 / Published: 30 June 2020
(This article belongs to the Section Cancer Pathophysiology)
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 × 109/L) and bleeding risk, the development and pathogenesis of acquired von Willebrand syndrome, and the clinical approach to this paradoxical scenario in ET. View Full-Text
Keywords: essential thrombocythemia; cytoreductive therapy; excessive thrombocytosis; acquired von Willebrand syndrome essential thrombocythemia; cytoreductive therapy; excessive thrombocytosis; acquired von Willebrand syndrome
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MDPI and ACS Style

Awada, H.; Voso, M.T.; Guglielmelli, P.; Gurnari, C. Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding. Cancers 2020, 12, 1746.

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