A 44-year-old male presents with the sudden onset of severe right upper quadrant (RUQ) abdominal pain, ascites, tender hepatomegaly, and hematemesis. These symptoms are suggestive of Budd-Chiari syndrome, a disorder that is caused by
Correct Answer: Thrombosis of the hepatic vein
Description: Abnormalities of the hepatic blood flow occur in various disease states and result in characteristic symptoms. Because of their dual blood supply, aerial occlusion of either the hepatic aery or the poal vein rarely results in liver infarcts. However, thrombosis of branches of the hepatic aery may result in a pale (anemic) infarct, or possibly a hemorrhagic infarct due to blood flow from the poal vein. In contrast, occlusion of the poal vein, which may be caused by cirrhosis or malignancy, may result in a wedge-shaped red area called an infarct of Zahn. This is a misnomer, however, since it is not really an infarction but instead is the result of focal sinusoidal congestion. Hepatic vein thrombosis (Budd-Chiari syndrome) is associated with polycythemia vera, pregnancy, and oral contraceptives. Clinically, Budd-Chiari syndrome is characterized by the sudden onset of severe right upper quadrant abdominal pain, ascites, tender hepatomegaly, and hematemesis. Occlusion of the central veins, called venoocclusive disease, may be rarely seen in Jamaican drinkers of alkaloid-containing bush tea, but is much more commonly found following bone marrow transplantation (up to 25% of allogenic marrow transplants). Reference: Robbins & Cotran Pathologic Basis of Disease, 9edition
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