NDT Advance Access published online on November 7, 2007
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfm488
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Clinical and histological responses of renal amyloidosis to high-dose melphalan supported by autologous stem cell transplantation
1Division of Molecular Medicine, Center for Translational and Advanced Animal Research, Tohoku University School of Medicine, Sendai, 2Department of Internal Medicine, Yamagata Prefectural Central Hospital, Yamagata, 3Division of Nephrology, Hypertension and Endocrinology, Department of Internal Medicine, Tohoku University Graduate School of Medicine, Sendai and 4Department of Pathology, Yamagata University School of Medicine, Yamagata, Japan
Correspondence and offprint requests to: Satoru Sanada, Division of Molecular Medicine, Center for Translational and Advanced Animal Research, Tohoku University School of Medicine, Seiryouchou 2-1, Aoba-ku, Sendai 980-0875 Japan. Email: sasanada@mail.tains.tohoku.ac.jp
Keywords: high-dose melphalan; primary amyloidosis; renal biopsy; stem cell transplantation
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| Introduction |
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Primary amyloidosis (AL) is a clonal plasma cell dyscrasia. Monoclonal immunoglobulin light or heavy chain fragments produced by abnormal plasma cells deposit in multiple organs, such as the kidneys, heart and gastrointestinal tract, resulting in severe multi-organ dysfunction. The principal clinical manifestation due to renal involvement is massive proteinuria, which leads to nephrotic syndrome and subsequent renal failure [1,2]. The therapeutic strategy for AL has progressed in parallel with that of multiple myeloma (MM), which is a malignant type of plasma cell disorder. Recently, high-dose chemotherapy with autologous stem cell transplantation (HDT/SCT) has been applied to MM, with a much better outcome than conventional therapy [3]. Thus, this therapy has been further applied to AL, and meta-analysis reveals that
50–75% of renal AL patients treated with HDT/SCT showed more than 50% reduction in proteinuria [4,5].
In spite of many reports
| Case |
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Clinical course until first renal biopsy
Pathological findings in first renal biopsy (Figure 1A and B)
HDT/SCT therapy and following clinical course
Pathological findings of second renal biopsy (Figure 1C and D)
Quantitative analysis of amyloid deposition in the glomeruli (Figure 2)
| Discussion |
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