Rima Shobar, MD, MS1, Deepanshu Jain, MD1, Bayarmaa Mandzhieva, MD1, Muhammad K. Hasan, MD2; 1Advent Health, Orlando, FL; 2AdventHealth Orlando, Orlando, FL
Introduction: Amyloidosis is a rare systemic disorder that results from extracellular deposits of insoluble fibrils of protein polymers that disrupt tissue structure and function. AL (immunoglobulin light chain) amyloidosis is the most common type and is associated with plasma cell dyscrasias. 12–30% of multiple myeloma (MM) patients have coexisting AL amyloidosis. We present a rare case of multiple myeloma presenting as obstructive jaundice caused by amyloidosis involving the ampulla of Vater.
Methods: A 79-year-old female with history of dementia presented with icterus, nausea, vomiting and a 30-pound unintentional weight loss over a few months. She had elevated LFTs (total bilirubin 2.2, alkaline phosphatase 358, ALT 158, and AST 347). On Exam, she was afebrile, icteric and the abdomen was benign. MRI revealed a dilated common bile duct (CBD) with no choledocholithiasis or mass in head of pancreas. EUS confirmed MRI findings and in addition showed an enlarged peri-duodenal lymph node. ERCP revealed a large duodenal ulcerated mass with a bulging major papilla. Cholangiogram revealed dilated common bile duct with ampullary stenosis. A biliary sphincterotomy was performed and a plastic stent was placed into the common bile duct. Histopathology of the ampullary biopsy showed pale eosinophilic material deposited in the duodenal mucosa in between glands and in walls of small blood vessels with features consistent with amyloidosis. Congo red stain confirmed the apple green-birefringent nature of amyloid deposits. Fine needle biopsy from the peri-duodenal lymph node also contained small amounts of similar eosinophilic material. Further work up established the diagnosis of multiple myeloma (MM) and systemic amyloidosis involving the heart and peripheral nervous system. Patient is currently receiving chemotherapy for MM. Discussion: Jaundice is a rare manifestation of MM and when present is usually due to hepatic infiltration. Obstructive jaundice secondary to amyloidosis of the ampulla of Vater is extremely rare with very few cases reported in the literature. This case highlights the wide range of clinical presentations of amyloidosis and the importance of keeping it all in differential during the work up and treatment.
Disclosures: Rima Shobar indicated no relevant financial relationships. Deepanshu Jain indicated no relevant financial relationships. Bayarmaa Mandzhieva indicated no relevant financial relationships. Muhammad Hasan indicated no relevant financial relationships.