Cause:Myeloproliferative diseases, esp P. vera, even when not clinically apparent; also PNH, antiphospholipid syndrome, and inherited deficiency of protein C, S, and antithrombin III, factor V Leiden mutation, and prothrombin gene mutation; birth control pill use
Pathophys:Acute or chronic occlusion of hepatic veins
Sx:Abdominal pain, hepatomegally, ascites in most. Nausea, vomiting, and icterus in acute
Si:Spenomegally and varices in chronic
r/o tricuspid regurgitation, constrictive pericarditis, and atrial myxoma; cholecystitis
Lab: Chem:AST and ALT increased markedly; alk phos and bilirubin elevated some
Xray:Ultrasound w Doppler, MRI, and CT all show the lesion