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Nephrology Dialysis Transplantation 2005 20(2):434-437; doi:10.1093/ndt/gfh545
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Nephrol Dial Transplant Vol. 20 No. 2 © ERA–EDTA 2005; all rights reserved

Case Report

Development of systemic {lambda}-light chain amyloidosis in a patient with {gamma}-heavy chain deposition disease during long-term follow-up

Atsushi Komatsuda1, Nobuki Maki1, Hideki Wakui1, Hiroshi Ohtani1, Takashi Hatakeyama1, Tadashi Yasuda2, Yasushi Nakamoto3, Hirokazu Imai4 and Ken-ichi Sawada1

1 Third Department of Internal Medicine, Akita University School of Medicine, 2 Department of Internal Medicine, Honjo Daiichi Hospital, Akita, 3 Department of Internal Medicine, Kichijoji Asahi Hospital, Tokyo and 4 Department of Nephrology and Rheumatology, Aichi Medical University, Aichi, Japan

Correspondence and offprint requests to: Atsushi Komatsuda, MD, Third Department of Internal Medicine, Akita University School of Medicine, 1-1-1 Hondo, Akita City, Akita 010-8543, Japan. Email: komatsud@med.akita-u.ac.jp

Keywords: AL amyloidosis; CH1-deleted {gamma}-heavy; heavy chain deposition disease; {lambda}-light chain

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



   Introduction
 
Heavy chain deposition disease (HCDD) is one of the renal monoclonal immunoglobulin deposition diseases, histologically characterized by the presence of nodular glomerulosclerosis and non-amyloidogenic deposits of monoclonal heavy chains without associated light chains [1]. In 1993, Aucouturier et al. [2] reported the first documented cases and defined the concept of HCDD. They suggested that the deletion of the first constant domain (CH1) of {gamma}-heavy chains is the hallmark of the disease. In 1992, Tubbs et al. [3] described two patients with nodular glomerulosclerosis with deposits of {gamma}-heavy chains unassociated with light chains, but used the term ‘pseudo-{gamma} heavy chain deposition disease’. In these patients, immunochemical analyses of abnormal IgG were not performed. Although >10 years have passed since its first documentation and definition of the concept of HCDD, reports of HCDD remain extremely rare [1 (and references therein),4–8]. The natural . . . [Full Text of this Article]



   Case
 


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