Recovery of acute renal failure and nephrotic syndrome following autologous stem cell transplantation for primary (AL) amyloidosis.

Primary AL amyloidosis is a plasma cell dyscrasia characterized by the deposition of monoclonal immunoglobulin light-chain protein. The latter forms insoluble fibrils with -pleated sheet configuration within a variety of tissues, resulting in severe organ dysfunction and poor outcome. In patients with primary AL amyloidosis, cyclic treatment with melphalan and prednisone improves by 2-fold median survival from 8 to 18 months [1]. However, this regimen affords no benefit on renal survival, while kidney involvement occurs in 48–82% of patients [1,2]. The most common renal manifestations include nephrotic-range proteinuria and progressive renal failure that ultimately require dialysis support in one-third of all cases [3]. To break down the production of the amyloidogenic immunoglobulin by the underlying B-cell clone and stop tissue deposition, dose-intensive melphalan with autologous blood stem cell support is currently under evaluation in primary AL amyloidosis [4]. We report the case of a 50-year-old woman with primary AL amyloidosis who experienced a complete recovery of both nephrotic syndrome and protracted anuric renal failure after high-dose melphalan and autologous blood stem cell transplantation (SCT). Case