Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report.
Journal
Pathologica
ISSN: 1591-951X
Titre abrégé: Pathologica
Pays: Italy
ID NLM: 0401123
Informations de publication
Date de publication:
Jun 2020
Jun 2020
Historique:
received:
13
01
2020
accepted:
20
04
2020
entrez:
8
8
2020
pubmed:
8
8
2020
medline:
19
8
2021
Statut:
ppublish
Résumé
Small hepatic veins Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this case only a liver biopsy can demonstrate the presence of a small vessels outflow block. Paroxysmal nocturnal haemoglobinuria (PNH) is one of the most severe acquired thrombophilic state and represents one of the main aetiological factors of Budd-Chiari syndrome. In patient affected by PNH with liver impairment and/or ascites, Budd-Chiari syndrome must be always taken into consideration and, if necessary, a liver biopsy performed to exclude the small hepatic veins involvement. We report a case of small hepatic veins Budd-Chiari syndrome secondary to paroxysmal nocturnal haemoglobinuria.
Identifiants
pubmed: 32760053
doi: 10.32074/1591-951X-4-20
pmc: PMC7931566
doi:
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
102-104Informations de copyright
Copyright © 2020 Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology.
Références
Blood. 2007 Dec 1;110(12):4123-8
pubmed: 17702897
Surg Case Rep. 2015;1(1):57
pubmed: 26366354
J Thromb Haemost. 2012 Jan;10(1):1-10
pubmed: 22077430
J Thromb Thrombolysis. 2014 May;37(4):536-9
pubmed: 23813023
Eur J Haematol. 2011 Dec;87(6):473-9
pubmed: 21883481
Hippokratia. 2013 Jan;17(1):81-4
pubmed: 23935352
Ther Clin Risk Manag. 2016 Aug 01;12:1161-70
pubmed: 27536121
Hematology Am Soc Hematol Educ Program. 2006;:24-8, 516
pubmed: 17124035