title: Clinical case of the month. A rare case of Budd Chiari syndrome.
abstract: Budd Chiari syndrome is a rare disorder resulting from hepatic venous outflow tract obstruction anywhere from the small hepatic veins to the suprahepatic inferior vena cava. This patient has a hypercoagulable state secondary to heterozygous mutation of factor V and the JAK2 mutation and is being anticoagulated. We hypothesize that the low protein C and low antithrombin III levels seen in this patient resulted from decreased synthetic function of the liver and were not indicative of actual deficiencies. Indeed, reports of coexisting protein C and antithrombin III deficiencies are not existent in the literature and likely are not compatible with life. All patients with BCS warrant a hypercoagulable work up and JAK2 mutation is increasingly recognized as a contributing factor, even in those patients without obvious signs of polycythemia vera.
authors.affiliation: Department of Medicine, Louisiana State University Health Sciences Center, New Orleans, USA.
authors.forename: Theepa
authors.initials: T
authors.lastname: Thayalakulasingam
authors.forename: Rehan
authors.initials: R
authors.lastname: Mohammed
authors.forename: Shibu
authors.initials: S
authors.lastname: Varughese
authors.forename: Arthur
authors.initials: A
authors.lastname: Zieske
authors.forename: David L
authors.initials: DL
authors.lastname: Smith
authors.forename: Lee S
authors.initials: LS
authors.lastname: Engel
authors.forename: Brian
authors.initials: B
authors.lastname: Boulmay
authors.forename: Fred A
authors.initials: FA
authors.lastname: Lopez
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use*; D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D014057:Tomography, X-Ray Computed; D014859:Warfarin / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D000925:Anticoagulants; D014859:Warfarin