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Systemic Amyloidosis Presenting as Budd-Chiari Syndrome: A Case Report
Naman Lodha1, Samarth Bhat K S1, Kartikeya Mathur2
1Department of General Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.
Insights
Systemic amyloidosis, a protein misfolding disorder, can rarely manifest as Budd-Chiari syndrome (BCS), a liver vein obstruction. This case highlights the importance of considering amyloid light chain amyloidosis in BCS diagnosis.
Area of Science:
- Hepatology
- Nephrology
- Cardiology
- Hematology
Background:
- Budd-Chiari syndrome (BCS) involves hepatic venous outflow obstruction, often linked to hypercoagulable states.
- Systemic amyloidosis is characterized by misfolded protein deposition causing end-organ damage and coagulation abnormalities.
- Amyloidosis frequently presents with bleeding diathesis, complicating its clinical picture.
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.
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