Systemic Amyloidosis Presenting as Budd-Chiari Syndrome: A Case Report.

Abstract

Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow tract obstruction and is commonly associated with an underlying hypercoagulable state. Systemic amyloidosis is a disorder characterized by systemic deposition of misfolded proteins leading to end organ damage. Amyloidosis is commonly associated with coagulation abnormalities, mainly leading to increased bleeding diathesis. Here, we report a case of amyloid light chain (AL) amyloidosis presenting as BCS. A 40-year-old man presented with abdominal distension along with anorexia and weight loss. On evaluation, he had severe hypoalbuminemia, raised alkaline phosphatase, and non-visualization of hepatic veins on abdominal imaging. Further evaluation confirmed the diagnosis of AL amyloidosis with renal, cardiac, and hepatic involvement. AL amyloidosis rarely can present with BCS. A high index of suspicion is needed as symptoms can be variable and non-specific.