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Nephrol Dial Transplant (2003) 18: 2175-2177
© 2003 European Renal Association-European Dialysis and Transplant Association

Case Report

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

Renaud Snanoudj1, Marie-France Mamzer-Bruneel2, Olivier Hermine3, Jean-Pierre Grünfeld1 and Dominique Chauveau1

1Nephrology Department, Hôpital Necker, Assistance Publique Hôpitaux de Paris, 2Intensive Care Unit, Hôpital Necker, Assistance Publique Hôpitaux de Paris and 3Haematology Department, Hôpital Necker, Assistance Publique Hôpitaux de Paris, France

Correspondence and offprint requests to: Dr Renaud Snanoudj, Nephrology Department, Hôpital de Bicêtre, 78 avenue du Général Leclerc, F-94275 Le Kremlin-Bicêtre, France. Email: rsnanoudj@aol.com

Keywords: acute renal failure; AL amyloidosis; autologous stem cell transplantation; nephrotic syndrome

The first 150 words of the full text of this article appear below.



   Introduction
 
Primary AL amyloidosis is a plasma cell dyscrasia characterized by the deposition of monoclonal immunoglobulin light-chain protein. The latter forms insoluble fibrils with ß-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 . . . [Full Text of this Article]



   Case
 


   Discussion
 

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[Abstract] [Full Text] [PDF]