Abstract
ABSTRACTWe report the case of a patient with an unusual acute Budd-Chiari syndrome (BCS). The patient presented with high-grade fever and right upper quadrant pain. Infiltrative lesions at the right hepatic lobe and segment IVB with intrahepatic inferior vena cava and right hepatic vein thrombus appeared on abdominal imaging. Liver biopsy revealed hepatic infarction compatible with acute BCS. Thrombophilia work-up demonstrated low protein C activity with the -1657C/T mutation of the PROC gene. Necrotic liver mass with acute BCS related to congenital protein C deficiency was diagnosed. Patient symptoms and necrotic masses improved after anticoagulant treatment for 4 months.
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