Budd-Chiari syndrome complicating essential thrombocythemia in an adolescent: favorable outcome of TIPS procedure.

Autor: Tantawy AA; Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt., Adly AA, Elhenawy YI
Jazyk: angličtina
Zdroj: Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis [Blood Coagul Fibrinolysis] 2015 Sep; Vol. 26 (6), pp. 691-4.
DOI: 10.1097/MBC.0000000000000325
Abstrakt: Budd-Chiari syndrome (BCS) is a liver disorder characterized by hepatic venous outflow obstruction, mainly resulting from thrombosis of the terminal part of the hepatic veins or the inferior vena cava. It causes hepatic congestion, ascites, portal hypertension, and collateral circulation between the obstructed and contiguous patent venous territories. BCS is reported complicating myeloproliferative disorders, as well other prothrombotic events. Essential thrombocythemia is one of the most frequent myeloproliferative disorders that cause BCS, and in some cases, it may be the initial presentation. Many treatment options have been proposed for BCS, routine anticoagulation therapy being recommended as the first therapeutic approach.
Databáze: MEDLINE