Primary amyloidosis (AL) presenting with nephrotic syndrome: a case report and discussion

Primary amyloidosis (AL) is a rare disorder with only eight cases per million a year. AL is a plasma cell disorder in which neoplastic clonal plasma cells in the bone marrow produce monoclonal immunoglobulin light chains that form protease resistant amyloid fibrils. AL fibrils accumulate within tissues systemically, causing progressive organ impairment to ultimate death. Median survival in AL with treatment is only 17 months depending upon variable prognostic factors. Most patients present with nephrotic syndrome and associated peripheral edema because the kidney is the most common organ involved in AL. This article describes the case of a patient who presented to City Hospital in Martinsburg, W.Va., and was diagnosed with primary amyloidosis, and reviews the current literature on this illness.