A 38-year-old man presents with abdominal distension, hepatomegaly, and ascites. He has a history of recurrent venous thrombosis. JAK2 V617F mutation is positive. Liver biopsy shows centrilobular congestion and sinusoidal dilatation. What is the underlying cause?
- A Hepatic veno-occlusive disease (VOD)
- B Right-sided heart failure
- C Budd-Chiari syndrome ✓
- D Constrictive pericarditis
Correct answer: C. Budd-Chiari syndrome
Explanation
Budd-Chiari syndrome is obstruction of the hepatic veins or inferior vena cava. Myeloproliferative neoplasms (polycythemia vera, essential thrombocythemia) account for the majority of cases, and JAK2 V617F is positive in ~40-55%. Liver biopsy shows centrilobular congestion and sinusoidal dilatation. VOD (sinusoidal obstruction syndrome) affects small venules and occurs post bone marrow transplant. Right heart failure and constrictive pericarditis cause passive congestion but are not thrombotic.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
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