BACKGROUND: The role of factor V Leiden as a cause of Budd-Chiari syndrome has only recently been described. AIMS: To assess the specific features of factor V Leiden related Budd-Chiari syndrome. PATIENTS: Sixty three consecutive patients with hepatic vein or terminal inferior vena cava thrombosis. METHODS: Standardised chart review. RESULTS: Factor V Leiden was found in 20 patients (31% (95% CI 20-43)). In the subgroup of patients with, compared with the subgroup without, factor V Leiden, a combination of prothrombotic states was more common (70% (95% CI 50-90) v 14% (95% CI 3-24)); inferior vena cava thrombosis was more frequent (40% (95% CI 19-61) v 7% (95% CI 0-14)); and distribution of initial alanine aminotransferase values was bimodal (almost normal or extremely increased) versus unimodal (p=0.003). Factor V Leiden accounted for four of five cases of massive ischaemic necrosis (transaminases >50-fold the upper limit of normal values) (p=0.014), and also for all three cases developing during pregnancy. Patients with and without factor V Leiden did not differ with regard to mortality, portosytemic shunting, or listing for liver transplantation. Hepatocellular carcinoma developed in two patients; both had factor V Leiden and indolent obstruction of the inferior vena cava. CONCLUSIONS: In patients with Budd-Chiari syndrome, factor V Leiden (a) is common; (b) precipitates thrombosis mostly when combined with another risk factor; (c) is associated with one of two contrasting clinical pictures: indolent thrombosis-particularly of the inferior vena cava-or massive ischaemic necrosis; and (d) is a major cofactor of Budd-Chiari syndrome developing during pregnancy.
BACKGROUND: The role of factor V Leiden as a cause of Budd-Chiari syndrome has only recently been described. AIMS: To assess the specific features of factor V Leiden related Budd-Chiari syndrome. PATIENTS: Sixty three consecutive patients with hepatic vein or terminal inferior vena cava thrombosis. METHODS: Standardised chart review. RESULTS:Factor V Leiden was found in 20 patients (31% (95% CI 20-43)). In the subgroup of patients with, compared with the subgroup without, factor V Leiden, a combination of prothrombotic states was more common (70% (95% CI 50-90) v 14% (95% CI 3-24)); inferior vena cava thrombosis was more frequent (40% (95% CI 19-61) v 7% (95% CI 0-14)); and distribution of initial alanine aminotransferase values was bimodal (almost normal or extremely increased) versus unimodal (p=0.003). Factor V Leiden accounted for four of five cases of massive ischaemic necrosis (transaminases >50-fold the upper limit of normal values) (p=0.014), and also for all three cases developing during pregnancy. Patients with and without factor V Leiden did not differ with regard to mortality, portosytemic shunting, or listing for liver transplantation. Hepatocellular carcinoma developed in two patients; both had factor V Leiden and indolent obstruction of the inferior vena cava. CONCLUSIONS: In patients with Budd-Chiari syndrome, factor V Leiden (a) is common; (b) precipitates thrombosis mostly when combined with another risk factor; (c) is associated with one of two contrasting clinical pictures: indolent thrombosis-particularly of the inferior vena cava-or massive ischaemic necrosis; and (d) is a major cofactor of Budd-Chiari syndrome developing during pregnancy.
Authors: M H Denninger; Y Chaït; N Casadevall; S Hillaire; M C Guillin; A Bezeaud; S Erlinger; J Briere; D Valla Journal: Hepatology Date: 2000-03 Impact factor: 17.425
Authors: M Bourlière; Y P Le Treut; D Arnoux; P Castellani; L Bordigoni; A Maillot; M Antoni; D Botta; B Pol; A P Gauthier Journal: Gut Date: 1990-08 Impact factor: 23.059
Authors: P Fickert; H Ramschak; L Kenner; G Hoefler; T A Hinterleitner; W Petritsch; M Klimpfinger; G J Krejs; R E Stauber Journal: Gastroenterology Date: 1996-12 Impact factor: 22.682
Authors: S Pelletier; B Landi; J C Piette; P Ekert; A Coutellier; C Desmoulins; J P Fadlallah; S Herson; D Valla Journal: J Hepatol Date: 1994-07 Impact factor: 25.083
Authors: J B Dilawari; P Bambery; Y Chawla; U Kaur; S R Bhusnurmath; H S Malhotra; G K Sood; S K Mitra; S K Khanna; B S Walia Journal: Medicine (Baltimore) Date: 1994-01 Impact factor: 1.889
Authors: J Schubert; M Alvarado; P Uciechowski; M Zielinska-Skowronek; M Freund; H Vogt; R E Schmidt Journal: Br J Haematol Date: 1991-11 Impact factor: 6.998
Authors: Demetrios N Samonakis; Ioannis E Koutroubakis; Aekaterini Sfiridaki; Niki Malliaraki; Pavlos Antoniou; John Romanos; Elias A Kouroumalis Journal: Dig Dis Sci Date: 2004-05 Impact factor: 3.199
Authors: Edyta Sutkowska; Robert D McBane; Alfonso J Tafur; Krzysztof Sutkowski; Diane E Grill; Joshua P Slusser; Waldemar E Wysokinski Journal: J Gastroenterol Date: 2012-12-18 Impact factor: 7.527