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Multiple Myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
The monoclonal protein itself can rarely have pathological activity. Rarely serum free light chain (usually lambda) is amyloidogenic leading to AL amyloidosis. Monoclonal gammopathy of renal significance are a range of renal disorders related to a small MGUS plasma cell clone. Associations for MGUS have also been found with a variety of skin disorders, peripheral neuropathies, and ocular lens deposition. The term monoclonal gammopathy of clinical significance (MGCS) refers to this group of disorders that are rare, poorly understood, and hence under-recognized.6
Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review
Published in Renal Failure, 2023
Li Zhu, Lei Wang, Hongxia Shi, Lei Jiang, Xin Li, Chunying Shao, Yu Yan, Bao Dong, Wanzhong Zou, Li Zuo
Our patient presented with renal insufficiency and renal glycosuria. Although serum creatinine was significantly elevated, uric acid and serum phosphorus were normal, and hypokalemia persisted, which suggests the presence of Fanconi syndrome. Urinary protein was mainly low molecular weight protein, and albumin was also present. It was considered that the lesions mainly involved the proximal renal tubules and glomerulus. Due to the presence of monoclonal immunoglobulin, monoclonal gammopathy of renal significance (MGRS) or MM was suspected. Renal biopsy and bone puncture confirmed our hypothesis. It is suggested that monoclonal immunoglobulin-induced crystalline nephrology should be confirmed when Fanconi syndrome is complicated with monoclonal immunoglobulin. If there is evidence of glomerular involvement at the same time, caution should be taken with LCCP.
A case of light chain deposition disease involving the kidney with a normal serum free kappa/lambda light chain ratio
Published in Renal Failure, 2022
Suojian Zhang, Haifeng Ni, Qin Xu, Xiaoqin Cai, Haitao Li, Zhiqiang Wei, Juan Cao
Light chain deposition disease (LCDD) is a monoclonal gammopathy-related disease, which can cause damage to many organs, including the liver, heart, lung, and kidney. The kidney is one of the most commonly affected organs [1–3]. Patients with LCDD involving the kidney may present with proteinuria, hematuria, nephrotic syndrome, and renal impairment [4]. LCDD can be secondary to multiple myeloma and plasma cell disease, or it may not be associated with any other disease. In 2012, the International Kidney and Monoclonal Gammopathy Research Group named the condition of monoclonal gammopathy without plasma cell disease or B lymphocyte proliferative disease, but with renal damage, as monoclonal gammopathy of renal significance (MGRS) [5]. LCDD is a rare disease and previously reported cases have had significantly increased or decreased serum free kappa/lambda ratios [1,6,7]. Here, we report a case of LCDD involving the kidney with a normal serum-free kappa/lambda ratio.
Monoclonal glomerulopathy with features of cryoglobulinemic glomerulopathy in murine multiple myeloma model
Published in Ultrastructural Pathology, 2020
Ping L. Zhang, Guillermo A. Herrera, Bei Liu
Multiple myeloma is a malignant proliferation of monoclonal plasma cells in the bone marrow, which causes 1% of cancer deaths and results in high morbidity from renal dysfunction due to the monoclonal immunoglobulin light chain deposition.1,2 Myeloma occurs mainly in the elderly. The mean ages for men and women to develop myeloma are 67 and 70 years old, respectively, without gender difference. Approximately 3% to 5% of individuals older than 50 have a monoclonal gammopathy of undetermined significance (MGUS) in absence of clinical evidence of myeloma. Some may progress and deposit monoclonal immunoglobulins in the kidney causing significant injury to glomeruli, renal tubules and/or renal vessels, so-called monoclonal gammopathy of renal significance.3–5 Good animal models are needed to investigate the etiology, disease progressions, and treatment effects in approximately 10 variants of the para-protein-related renal diseases.