Literature DB >> 25410550

A case of endocapillary proliferative glomerulonephritis with macrophages phagocytosing monoclonal immunoglobulin lambda light chain.

Hirofumi Watanabe1, Yutaka Osawa, Shin Goto, Masato Habuka, Naofumi Imai, Yumi Ito, Takayuki Hirose, Takaaki Chou, Ryuji Ohashi, Akira Shimizu, Takashi Ehara, Takashi Shimotori, Ichiei Narita.   

Abstract

Multiple myeloma (MM) is a plasma-cell neoplasm that can cause renal disorders. Renal lesions in MM can present with a very rare pathological manifestation involving a specific monoclonal immunoglobulin (Ig). We report the case of a 33-year-old woman who had edema, fatigue, elevated serum creatinine levels, hypoalbuminemia, and hypercholesterolemia. She had persistent hematuria and proteinuria lasting 3 years. Serum protein electrophoresis showed an M-spike, and serum immunofixation demonstrated the presence of monoclonal IgG λ. She had proteinuria in the nephrotic range, and a monoclonal λ fragment was present on urine immunofixation. Renal biopsy showed proliferative glomerulonephritis with λ light chain and C3c deposition and inflammatory cell infiltration with CD68. Macrophage lysosomes contained λ light chains, suggesting their partial phagocytosis. She was diagnosed with symptomatic MM and was treated with bortezomib and dexamethasone and an autologous peripheral stem cell transplant conditioned with intravenous melphalan. She achieved a partial response with decreased serum monoclonal protein and improved renal function. This case may be categorized as a monoclonal gammopathy-associated proliferative glomerulonephritis. The biopsy finding of partially phagocytosed Ig λ light chains by macrophages is very rare; this pathological condition is similar to crystal-storing histiocytosis.
© 2014 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.

Entities:  

Keywords:  crystal-storing histiocytosis; endocapillary hypercellularity; membranoproliferative glomerulonephritis; monoclonal gammopathy-associated proliferative glomerulonephritis; multiple myeloma

Mesh:

Substances:

Year:  2014        PMID: 25410550     DOI: 10.1111/pin.12229

Source DB:  PubMed          Journal:  Pathol Int        ISSN: 1320-5463            Impact factor:   2.534


  4 in total

Review 1.  An update of proliferative glomerulonephritis with monoclonal immunoglobulin deposits.

Authors:  Manna Li; Gaosi Xu
Journal:  Clin Kidney J       Date:  2021-12-14

2.  Successful treatment with bortezomib and dexamethasone for proliferative glomerulonephritis with monoclonal IgG deposits in multiple myeloma: a case report.

Authors:  Rio Noto; Nozomu Kamiura; Yuichiro Ono; Sumie Tabata; Shigeo Hara; Hideki Yokoi; Akihiro Yoshimoto; Motoko Yanagita
Journal:  BMC Nephrol       Date:  2017-04-06       Impact factor: 2.388

3.  [Advances in diagnosis and treatment of monoclonal immunoglobulin deposit disease].

Authors:  J T Zhao; W L Ye; J L Zhuang
Journal:  Zhonghua Xue Ye Xue Za Zhi       Date:  2020-01-14

4.  An Unusual Case of Lysosomal Paraprotein Accumulation in Glomerular Endothelial Cells.

Authors:  Sekiko Taneda; Kazuho Honda; Shigeru Horita; Kosei Matsue; Yoshiaki Usui; Michihiro Mitobe; Shota Ogura; Kosaku Nitta; Hideaki Oda
Journal:  Kidney Int Rep       Date:  2019-10-01
  4 in total

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