Monoclonal light chain crystalline podocytopathy and tubulopathy associated with monoclonal gammopathy of renal significance: a case report and literature review. 2018

Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
Renal Division, Department of Medicine, Peking University First Hospital, Beijing, 100034, People's Republic of China.

Monoclonal gammopathy of renal significance (MGRS) is a recently defined group of renal diseases caused by monoclonal immunoglobulin secreted by nonmalignant proliferative B cell or plasma cell. Monoclonal immunoglobulin can form different types of structures deposited in renal tissue, including fibrils, granules, microtubules, crystals and casts, and has mostly been reported in multiple myeloma patients. Here we report a rare case with κ light chain crystals in both podocytes and tubular epithelial cells associated with MGRS, which adds more information to the spectrum of MGRS-related renal diseases. A 53-year old woman presented with albumin-predominant moderate proteinuria and renal failure. She had monoclonal IgGκ in the serum and monoclonal IgGκ plus free κ in the urine. Multiple myeloma and lymphoproliferative disorders were excluded. Renal biopsy confirmed κ-restricted crystal-storing renal disease involving the podocytes and proximal tubular epithelial cells. The patient was treated with bortezomib followed by lenalidomide-based chemotherapy, and renal function was stable after 1 year of follow-up. This is a rare case of combined crystalline podocytopathy and tubulopathy associated with MGRS, in which diagnosis was dependent on electron and immuno-electron microscopy.

UI MeSH Term Description Entries
D007147 Immunoglobulin Light Chains Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. There are two major types of light chains, kappa and lambda. Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule. Ig Light Chains,Immunoglobulins, Light-Chain,Immunoglobulin Light Chain,Immunoglobulin Light-Chain,Light-Chain Immunoglobulins,Chains, Ig Light,Chains, Immunoglobulin Light,Immunoglobulins, Light Chain,Light Chain Immunoglobulins,Light Chain, Immunoglobulin,Light Chains, Ig,Light Chains, Immunoglobulin,Light-Chain, Immunoglobulin
D007674 Kidney Diseases Pathological processes of the KIDNEY or its component tissues. Disease, Kidney,Diseases, Kidney,Kidney Disease
D007687 Kidney Tubules, Proximal The renal tubule portion that extends from the BOWMAN CAPSULE in the KIDNEY CORTEX into the KIDNEY MEDULLA. The proximal tubule consists of a convoluted proximal segment in the cortex, and a distal straight segment descending into the medulla where it forms the U-shaped LOOP OF HENLE. Proximal Kidney Tubule,Proximal Renal Tubule,Kidney Tubule, Proximal,Proximal Kidney Tubules,Proximal Renal Tubules,Renal Tubule, Proximal,Renal Tubules, Proximal,Tubule, Proximal Kidney,Tubule, Proximal Renal,Tubules, Proximal Kidney,Tubules, Proximal Renal
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010265 Paraproteinemias A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. These cells frequently secrete a structurally homogeneous immunoglobulin (M-component) and/or an abnormal immunoglobulin. Gammapathy, Monoclonal,Gammopathy, Monoclonal,Monoclonal Gammopathies,Paraimmunoglobulinemia,Paraimmunoglobulinemias,Paraproteinemia,Plasma Cell Dyscrasias,Monoclonal Gammapathies,Monoclonal Gammopathy,Cell Dyscrasia, Plasma,Dyscrasia, Plasma Cell,Monoclonal Gammapathy,Plasma Cell Dyscrasia
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D050199 Podocytes Highly differentiated epithelial cells of the visceral layer of BOWMAN CAPSULE of the KIDNEY. They are composed of a cell body with major CELL SURFACE EXTENSIONS and secondary fingerlike extensions called pedicels. They enwrap the KIDNEY GLOMERULUS capillaries with their cell surface extensions forming a filtration structure. The pedicels of neighboring podocytes interdigitate with each other leaving between them filtration slits that are bridged by an extracellular structure impermeable to large macromolecules called the slit diaphragm, and provide the last barrier to protein loss in the KIDNEY. Visceral Epithelial Cells, Glomerular,Epithelial Cells, Glomerular Visceral,Glomerular Visceral Epithelial Cells,Podocyte

Related Publications

Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
January 2023, Clinical nephrology,
Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
November 2022, Journal of nephrology,
Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
March 2020, Jornal brasileiro de nefrologia,
Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
June 2020, BMJ case reports,
Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
August 2021, Histopathology,
Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
May 2024, Kidney medicine,
Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
April 2020, Clinical nephrology,
Xiao-Juan Yu, and Xu-Jie Zhou, and Su-Xia Wang, and Fu-de Zhou, and Ming-Hui Zhao
September 2018, Medicine,
Copied contents to your clipboard!