A case of proliferative glomerulonephritis with immunoglobulin A1-lambda deposits successfully treated by chemotherapy
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CASE REPORT
A case of proliferative glomerulonephritis with immunoglobulin A1‑lambda deposits successfully treated by chemotherapy Yasuo Kusunoki1 · Tomoko Namba‑Hamano2 · Tsunayuki Kakimoto3 · Satoko Yamamoto1 · Natsuko Ikeda1 · Keiko Wakabayashi1 · Kumie Teramoto1 · Masanobu Takeji1 Received: 4 October 2019 / Accepted: 17 April 2020 © Japanese Society of Nephrology 2020
Abstract A 74-year-old man presented with nephrotic syndrome and kidney insufficiency. Laboratory tests revealed monoclonal gammopathy of immunoglobulin A-lambda. Renal biopsy revealed diffuse mesangial proliferation and double-contoured basement membranes. Immunofluorescent analyses showed granular deposition of immunoglobulin A and C3 at the capillary walls and mesangial regions. Immunohistochemistry suggested monoclonal deposition of immunoglobulin A1-lambda. Electron microscopic analyses showed finely granular electron-dense deposits at mesangial and subendothelial areas. These findings suggested immunoglobulin A-type proliferative glomerulonephritis with monoclonal immunoglobulin deposits. Based on the results of bone marrow aspiration, multiple myeloma was diagnosed. Because the renal manifestation was considered to be affected by monoclonal gammopathy, chemotherapy was initiated rather than immunomodulatory therapy. Although bortezomib and dexamethasone proved ineffective, second chemotherapy with elotuzumab, lenalidomide, and dexamethasone was successful, and kidney function recovered. Effective treatments for proliferative glomerulonephritis with monoclonal immunoglobulin deposits have not been established. This represents the first description of a patient successfully treated for proliferative glomerulonephritis with monoclonal immunoglobulin deposits by chemotherapy using elotuzumab. Keywords PGNMID · IgA · MPGN · Elotuzumab
Introduction Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) was first reported by Nasr et al. [1], and its pathological characteristics could not be assigned to any conventional conditions related to monoclonal immunoglobulin (Ig) G deposition, such as light- and heavy-chain deposition disease (LHCDD), cryoglobulinemic Electronic supplementary material The online version of this article (https://doi.org/10.1007/s13730-020-00480-y) contains supplementary material, which is available to authorized users. * Masanobu Takeji [email protected] 1
Division of Nephrology, Department of Internal Medicine, Toyonaka Municipal Hospital, 4‑14‑1, Shibahara‑cho, Toyonaka, Osaka 560‑8565, Japan
2
Department of Nephrology, Osaka University Graduate School of Medicine, Osaka, Japan
3
Division of Hematology, Department of Internal Medicine, Toyonaka Municipal Hospital, Osaka, Japan
glomerulonephritis, immunotactoid glomerulonephritis, and amyloidosis. In PGNMID, immunostaining reveals that the glomerular deposits are monoclonal, staining for a singlelight-chain isotype and a single heavy-chain subclass. On light microscopy (LM), membranoproliferative and diffuse prolifer
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