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Budd-Chiari syndrome (BCS) occurs when there is hepatic venous outflow obstruction. Chronic BCS may result in liver cirrhosis due to long-standing obstruction and tend to present late. We present the first case of BCS secondary to Janus tyrosine kinase 2 (JAK2) mutation resulting in "pseudocirrhosis" rather than cirrhosis of the liver. Pseudocirrhosis clinically and radiologically mimics cirrhosis without the classical histopathological changes, and it is usually associated with metastatic cancers.

Pseudocirrhosis in Chronic Budd Chiari Syndrome With Janus Tyrosine Kinase 2 (JAK2) Mutation