Budd Chiari Syndrome
Alpha 1 Antitrypsin deficiency
Ans:A.)Budd Chiari Syndrome.
Image shows:Budd-Chiari syndrome. Thrombosis of the major hepatic veins has caused profound hepatic congestion.
Hepatic Vein Thrombosis (Budd-Chiari Syndrome) .
- The Budd-Chiari syndrome results from the thrombosis one or more major hepatic veins and is characterized by hepatomegaly, ascites, and abdominal pain.
- Hepatic vein thrombosis is associated with myeloproliferative disorders (especially polycythemia vera), pregnancy, the postpartum state, the use of oral contraceptives, paroxysmal nocturnal hemoglobinuria, and intra-abdominal cancers, particularly hepatocellular carcinoma.
- With acutely developing thrombosis of the major hepatic veins or inferior vena cava, the liver is swollen and red-purple, with a tense capsule .
- On microscopic examination, severe centrilobular congestion and necrosis are apparent in affected hepatic parenchyma. In instances in which the thrombosis develops more slowly, centrilobular fibrosis is seen.
- The major veins may contain completely or incompletely occlusive fresh thrombi or, in chronic cases, organized adherent thrombi.