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Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area

The term splanchnic vein thrombosis encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis. Risk factors can be local or systemic. A local precipitating factor is rare in BCS, while it is common in patients with portal vein thrombosis. Ch...

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Autores principales: De Stefano, Valerio, Za, Tommaso, Ciminello, Angela, Betti, Silvia, Rossi, Elena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Università Cattolica del Sacro Cuore 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3248340/
https://www.ncbi.nlm.nih.gov/pubmed/22220260
http://dx.doi.org/10.4084/MJHID.2011.063
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author De Stefano, Valerio
Za, Tommaso
Ciminello, Angela
Betti, Silvia
Rossi, Elena
author_facet De Stefano, Valerio
Za, Tommaso
Ciminello, Angela
Betti, Silvia
Rossi, Elena
author_sort De Stefano, Valerio
collection PubMed
description The term splanchnic vein thrombosis encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis. Risk factors can be local or systemic. A local precipitating factor is rare in BCS, while it is common in patients with portal vein thrombosis. Chronic myeloproliferative neoplasms (MPN) are the leading systemic cause of splanchnic vein thrombosis, and are diagnosed in half BCS patients and one-third of EHPVO patients; the somatic mutation JAK2 V617F is detectable in a large majority of patients with overt MPN, and up to 40% of patients without overt MPN. Inherited thrombophilia is present in at least one-third of patients, and the factor V Leiden or the prothrombin G20210A mutations are the most common mutations found in BCS or EHPVO patients, respectively. Multiple factors are present in approximately one-third of patients with BCS and two- thirds of patients with portal vein thrombosis. In a few patient series from the Southern Mediterranean area the high prevalence of MPN and thrombophilia as underlying cause of BCS is confirmed, although the data should be considered preliminary. Peculiar risk factors present in the area are Behçet’s disease and hydatidosis; moreover, membraneous webs, typically found in Asian patients, are present in a significant portion of cases.
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spelling pubmed-32483402012-01-04 Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area De Stefano, Valerio Za, Tommaso Ciminello, Angela Betti, Silvia Rossi, Elena Mediterr J Hematol Infect Dis Review Articles The term splanchnic vein thrombosis encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis. Risk factors can be local or systemic. A local precipitating factor is rare in BCS, while it is common in patients with portal vein thrombosis. Chronic myeloproliferative neoplasms (MPN) are the leading systemic cause of splanchnic vein thrombosis, and are diagnosed in half BCS patients and one-third of EHPVO patients; the somatic mutation JAK2 V617F is detectable in a large majority of patients with overt MPN, and up to 40% of patients without overt MPN. Inherited thrombophilia is present in at least one-third of patients, and the factor V Leiden or the prothrombin G20210A mutations are the most common mutations found in BCS or EHPVO patients, respectively. Multiple factors are present in approximately one-third of patients with BCS and two- thirds of patients with portal vein thrombosis. In a few patient series from the Southern Mediterranean area the high prevalence of MPN and thrombophilia as underlying cause of BCS is confirmed, although the data should be considered preliminary. Peculiar risk factors present in the area are Behçet’s disease and hydatidosis; moreover, membraneous webs, typically found in Asian patients, are present in a significant portion of cases. Università Cattolica del Sacro Cuore 2011-12-19 /pmc/articles/PMC3248340/ /pubmed/22220260 http://dx.doi.org/10.4084/MJHID.2011.063 Text en This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Articles
De Stefano, Valerio
Za, Tommaso
Ciminello, Angela
Betti, Silvia
Rossi, Elena
Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area
title Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area
title_full Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area
title_fullStr Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area
title_full_unstemmed Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area
title_short Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area
title_sort causes of adult splanchnic vein thrombosis in the mediterranean area
topic Review Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3248340/
https://www.ncbi.nlm.nih.gov/pubmed/22220260
http://dx.doi.org/10.4084/MJHID.2011.063
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