Amyloidosis: A Rare Cause of Rapidly Progressive Liver Failure

Systemic light chain (AL) amyloidosis is a rare plasma cell dyscrasia characterized by the deposition of insoluble amyloid fibrils derived from immunoglobulin light chains in various organs. Hepatic involvement is common. However, the clinical presentation is often subtle or asymptomatic, with common findings including hepatomegaly and mild elevations in alkaline phosphatase. The progression to liver failure is rare but highly fatal complication of hepatic amyloidosis. We report a unique and severe case of a 63-year-old woman from China with jaundice and hepatomegaly. Her clinical course was notable for its rapid and aggressive progression to liver failure over a short period. A comprehensive diagnostic workup, including serum free light chain assays, which showed an abnormal kappa/lambda ratio, and a confirmatory liver biopsy demonstrating Congo red-positive amyloid deposits with apple-green birefringence under polarized light, established the diagnosis of systemic AL amyloidosis. No other obvious cause for her liver failure was identified. Intensive supportive care was initiated to manage the complications of liver dysfunction. The patient refused to started chemotherapy. As a result, her condition deteriorated rapidly and she succumbed to multi-organ failure. This case underscores the critical importance of considering AL amyloidosis in the differential diagnosis of patients presenting with unexplained jaundice and hepatomegaly, even in the absence of a known pre-existing hematologic disorder. The case emphasizes the need for a high index of suspicion, prompt diagnosis, and early, aggressive intervention to manage the underlying plasma cell disorder, as delayed recognition can lead to irreversible and catastrophic liver failure.