Sa1013 Lambda Light Chain Myeloma-Related Hepatic Amyloidosis: A Rare Etiology of Fatal Fulminant Liver Failure

Myeloma-related amyloid light chain (AL) systemic amyloidosis with hepatic-predominant manifestations is a rare condition. When present, hepatic amyloidosis is usually clinically silent. To date, there have been only 5 case reports of myeloma-related primary hepatic amyloidosis that progressed to fulminant hepatic failure. All of these cases were associated with kappa light chain. To the best of our knowledge, our case is the first report of fatal fulminant liver failure from lambda light chain myeloma-related hepatic amyloidosis. A 61year-old African American man with no significant past medical history presented with a 4-month history of worsening abdominal pain and intermittent syncopal episodes. Physical examination revealed orthostatic hypotension, hepatomegaly, and leg edema. Laboratory work up revealed alkaline phosphatase (ALP) of 945 IU/L, gamma glutamyl transferase of 1532 IU/L, aspartate aminotransferase of 77 IU/L, alanine aminotransferase of 89 IU/L, albumin of 1.2 g/dL, and nephrotic range proteinuria (5.6 g). Other results including total bilirubin (TB), creatinine, complete blood count, HIV antibody, alpha fetoprotein, CA199, angiotensin converting enzyme, iron studies, and serologic markers of viral hepatitis were all unremarkable. MRI of the abdomen revealed non-cirrhotic hepatomegaly of 20 cm with diffuse heterogenous enhancement, a normal sized spleen, a normal biliary system, esophageal and gastric varices. A transjugular liver biopsy revealed a hepatic-venous pressure gradient of 7 mmHg. Histology showed atrophic hepatocytes and extensive amyloid deposition. A bone marrow biopsy showed an amyloid-filled hypocellular marrow with 20% plasma cell dyscrasia, diagnostic of Myeloma-related AL systemic amyloidosis. Serum and urine protein electrophoresis showed a gamma monoclonal band with positive lambda chain. The free light chain kappa to lambda ratio was 0.05. He was discharged with plans to begin chemotherapy. However, two days after his discharge, he presented to a local hospital with altered mental status. He developed hyperbilirubinemia (TB of 10.4 mg/dL), coagulopathy (INR of 3.9), acute renal failure (creatinine of 5.8 mg/dL), and fluctuating encephalopathy. He rapidly deteriorated and passed away. Our case has highlighted many unique features of hepatic amyloidosis, including an atypical presentation of progressive abdominal pain, non-cirrhotic portal hypertension and rapid progression to acute liver and kidney failure. Even though the difference between the type of light chain involved may have prognostic value, but their clinical significance remains unknown. Hepatic amyloidosis should be considered in patients with chronic abdominal pain and marked elevation of ALP, especially if orthostatic hypotension and proteinuria are also present.