With diuretic therapy Mr. P.’s edema improved, but he then developed upper gastrointestinal bleed and became hypotensive, requiring blood transfusion to stabilize his hemodynamic state.

Urgent gastroscopy revealed a fresh gastric ulcer and grade 2 esophageal varices without evidence of active bleeding. He received intravenous proton pump inhibitor therapy, and Coumadin was stopped because of active bleeding. He became anuric and developed acute renal failure secondary to ischemic acute tubular necrosis, likely secondary to prolonged hemodynamic instability after GI bleed. His creatinine increased to over 500 in 3 days and required dialytic support. The serum immunoelectrophoresis results came back and showed monoclonal band of IgG Kappa.

At this time, as the Coumadin was withheld because of the GI bleed, it was decided to perform all invasive investigations before resuming anticoagulation. He underwent a bone marrow, liver and kidney biopsy while in hospital and off anticoagulation. The bone marrow showed 20% plasma cells, and the possibility of plasma cell dyscrasia or amyloidosis was raised. The kidney biopsy confirmed diagnosis of primary AL amyloidosis with positive Congo red staining. The hepatic dysfunction was of cholestatic type and liver biopsy showed advanced hepatic amyloidosis. His renal function improved after 2 weeks and he’s now off dialysis. He’s receiving chemotherapy with melphalan and decadron for primary AL amyloidosis. The prognosis is poor, but in younger patients stem cell transplant is now an option. Mr. P. is currently being referred for consideration of stem cell transplant.

This case illustrates the heroic course of a patient who initially presented with essentially asymptomatic worsening of lipids. Within 6 weeks, by the time he was undergoing investigations for nephrotic syndrome, he had developed a full-blown nephrotic syndrome and other complications. The underlying cause was rare and very unexpected — primary AL amyloidosis.