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

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...

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Detalles Bibliográficos
Autores principales: Awada, Hassan, Voso, Maria Teresa, Guglielmelli, Paola, Gurnari, Carmelo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7407619/
https://www.ncbi.nlm.nih.gov/pubmed/32629973
http://dx.doi.org/10.3390/cancers12071746
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author Awada, Hassan
Voso, Maria Teresa
Guglielmelli, Paola
Gurnari, Carmelo
author_facet Awada, Hassan
Voso, Maria Teresa
Guglielmelli, Paola
Gurnari, Carmelo
author_sort Awada, Hassan
collection PubMed
description 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(9)/L) and bleeding risk, the development and pathogenesis of acquired von Willebrand syndrome, and the clinical approach to this paradoxical scenario in ET.
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spelling pubmed-74076192020-08-12 Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding Awada, Hassan Voso, Maria Teresa Guglielmelli, Paola Gurnari, Carmelo Cancers (Basel) Review 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(9)/L) and bleeding risk, the development and pathogenesis of acquired von Willebrand syndrome, and the clinical approach to this paradoxical scenario in ET. MDPI 2020-06-30 /pmc/articles/PMC7407619/ /pubmed/32629973 http://dx.doi.org/10.3390/cancers12071746 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Awada, Hassan
Voso, Maria Teresa
Guglielmelli, Paola
Gurnari, Carmelo
Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding
title Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding
title_full Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding
title_fullStr Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding
title_full_unstemmed Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding
title_short Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding
title_sort essential thrombocythemia and acquired von willebrand syndrome: the shadowlands between thrombosis and bleeding
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7407619/
https://www.ncbi.nlm.nih.gov/pubmed/32629973
http://dx.doi.org/10.3390/cancers12071746
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