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Primary systemic amyloidosis: a case report

Jayashankar CA, D. S. Somasekar, Pavan Kumar Perugu, Santosh KV, Manjunath Nandennavar, Praveen Mathew.

Abstract
Primary amyloidosis is a group of monoclonal plasma cell disorders, characterized by extracellular deposition of immunoglobulin light chain fibrils in multiple organs leading to progressive multiorgan dysfunction. It is a rare disease which usually occurs in elderly persons and has a poor prognosis. We report a 72-year-old male patient with chronic abdominal distension, bilateral pitting pedal edema with nephrotic range proteinuria and amyloid deposition in liver and bone marrow. Immunoelectrophoresis of serum demonstrated the presence of immunoglobulin light chains of the circulating monoclonal protein. He was treated with IV bortezomib and IV dexamethasone.

Key words: AL Amyloidosis, Proteinuria, Hepatomegaly



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