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Clinical and Experimental Nephrology
Published in: Clinical and Experimental Nephrology 5/2010

01-10-2010 | Case Report

AL amyloidosis with IgD-lambda monoclonal gammopathy and lambda-type Bence-Jones protein: successful treatment by autologous stem cell transplantation

Authors: Chanhyok Sakurai-Chin, Yoshifumi Ubara, Tatsuya Suwabe, Junichi Hoshino, Tomoki Yonaha, Eiko Hasegawa, Keiichi Sumida, Rikako Hiramatsu, Masayuki Yamanouchi, Noriko Hayami, Junji Yamauchi, Naoyuki Tominaga, Naoki Sawa, Fumi Takemoto, Kazuhiro Masuoka, Kenmei Takaichi, Kenichi Oohashi

Published in: Clinical and Experimental Nephrology | Issue 5/2010

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Abstract

A 45-year-old Japanese woman had been diagnosed with monoclonal gammopathy of undetermined significance (MGUS) featuring urinary Bence-Jones protein of the lambda type (BJP-lambda) for 11 years. She then developed eyelid purpura, dyspnea, and flank pain. Abdominal CT scans revealed renal infarction. Biopsy of the kidney, heart, jejunum, and skin demonstrated amyloid deposits in the vessel walls, but not in the glomeruli. She was diagnosed as having AL amyloidosis with IgD-lambda monoclonal gammopathy and BJP-lambda. Autologous stem cell transplantation (SCT) was done after chemotherapy with vincristine, daunorubicin, dexamethasone (VAD), and high-dose melphalan (HDM). This reduced the IgD level from 156 to 0.1 mg/dL, along with the disappearance of BJP, despite cerebral infarction during chemotherapy. We recommend SCT for patients with IgD-associated AL amyloidosis.
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Metadata
Title
AL amyloidosis with IgD-lambda monoclonal gammopathy and lambda-type Bence-Jones protein: successful treatment by autologous stem cell transplantation
Authors
Chanhyok Sakurai-Chin
Yoshifumi Ubara
Tatsuya Suwabe
Junichi Hoshino
Tomoki Yonaha
Eiko Hasegawa
Keiichi Sumida
Rikako Hiramatsu
Masayuki Yamanouchi
Noriko Hayami
Junji Yamauchi
Naoyuki Tominaga
Naoki Sawa
Fumi Takemoto
Kazuhiro Masuoka
Kenmei Takaichi
Kenichi Oohashi
Publication date
01-10-2010
Publisher
Springer Japan
Published in
Clinical and Experimental Nephrology / Issue 5/2010
Print ISSN: 1342-1751
Electronic ISSN: 1437-7799
DOI
https://doi.org/10.1007/s10157-010-0311-2

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