Non‐crystalline light chain proximal tubulopathy without renal Fanconi syndrome in a patient with IgD‐λ multiple myeloma

2021 ◽  
Author(s):  
Daisuke Ikeda ◽  
Kosei Matsue
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Fanconi Syndrome ◽  
Renal Fanconi Syndrome ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy

scholarly journals Remission of light chain proximal tubulopathy in IgG λ-type multiple myeloma by lenalidomide and dexamethasone therapy

2019 ◽  
Vol 8 (3) ◽  
pp. 159-165
Author(s):  
Shinya Kawamoto ◽  
Yuji Hidaka ◽  
Yu Kaneko ◽  
Hideo Misawa ◽  
Katsuhiro Nagahori ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Dexamethasone Therapy ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy

Light chain proximal tubulopathy with cast nephropathy in monoclonal gammopathy of renal significance

2020 ◽  
Vol 13 (6) ◽  
pp. e234361
Author(s):  
Rebaika Chopra ◽  
Rosalba Santana de Roberts ◽  
Ibrahim Batal ◽  
Sachin Batra ◽  
Belinda Jim
Keyword(s):  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Fanconi Syndrome ◽  
Cell Damage ◽  
Biochemical Properties ◽  
Light Chains ◽  
Immunoglobulin Light Chains ◽  
Cast Nephropathy ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy

Kidney tubular disorders due to monoclonal immunoglobulin light chains are common manifestations of B-cell neoplasm. Cast nephropathy (CN) is the most frequent type of these disorders and may present with acute kidney injury (AKI) due to the presence of excess light chains in the distal tubules. Light chain proximal tubulopathy (LCPT) is an uncommon form of renal disease and may present as Fanconi syndrome due to proximal tubular cell damage by intracellular deposition of light chains. The concomitant disorder of both CN and LCPT is rare given the inherent differences in the biochemical properties of the immunoglobulin light chains of each disorder. We report a 64-year-old man who presented with AKI and Fanconi syndrome who was discovered to have both CN and LCPT due to the underlying disorder of monoclonal gammopathy of renal significance and who has responded favourably with conventional chemotherapy. We also review the existing literature on this interesting subject.

scholarly journals Light chain proximal tubulopathy with cast nephropathy in a case of multiple myeloma

2015 ◽  
Vol 25 (2) ◽  
pp. 119 ◽  
Author(s):  
KK Gowda ◽  
K Joshi ◽  
R Nada ◽  
R Ramachandran ◽  
M Sachdeva
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Cast Nephropathy ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy

scholarly journals Light Chain Proximal Tubulopathy: Expanding the Pathologic Spectrum with and without Deposition of Crystalline Inclusions

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Shree G. Sharma ◽  
Steven M. Bonsib ◽  
Didier Portilla ◽  
Ashutosh Shukla ◽  
Adam B. Woodruff ◽  
...  
Keyword(s):  
Electron Microscopy ◽  
Multiple Myeloma ◽  
Renal Disease ◽  
Physicochemical Properties ◽  
Light Chain ◽  
Proximal Tubules ◽  
Light Chains ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy ◽  
Crystal Accumulation

Light chain proximal tubulopathy (LCPT) is an uncommon form of renal disease associated with dysproteinemias. It is characterized by intracytoplasmic deposition of crystallized mostly kappa monoclonal light chains in proximal tubules (PTs). Crystals are located within lysosomes by electron microscopy (EM). Rare lambda LCPT cases without crystals by EM were described. Retrospectively, we reviewed clinical, light microscopic (LM), immunofluorescence (IF), and EM findings in 9 cases) (8 males, 1 female; mean age 57 years (38–81)) with multiple myeloma. LM showed abundant cytoplasmic droplets in PT cells in all cases. Droplets were also present in the podocytes, endothelial and parietal cells in one case. IF revealed staining of crystals with kappa in 3 and lambda in 6. EM showed electron dense rectangular, rhomboid, or needle shaped crystals in PT cells in 3 cases (33%), one of which had crystals in podocytes and interstitial cells. Six lambda LCPT cases showed no crystals by EM (67%). This may reflect differences in the physicochemical properties of light chains. The mechanisms of crystal accumulation in these cells and the significance of this finding are unknown.

scholarly journals C3 glomerulonephritis along with light chain proximal tubulopathy without crystal deposits in multiple myeloma: a case report

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Junhui Xu ◽  
Xiaojuan Yu ◽  
Suxia Wang ◽  
Miao Yan ◽  
Mangju Wang ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Renal Injury ◽  
Alternative Pathway ◽  
Complement C3 ◽  
C3 Glomerulonephritis ◽  
Serum Free Light Chain ◽  
Immunofixation Electrophoresis ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy

Abstract Background Multiple myeloma causes different types of renal injury. C3 glomerulonephritis (C3GN) is characterised by an abnormal deposition of complement C3 in the glomeruli due to abnormal activation of the alternative pathway of the complement system. While the association between C3GN and multiple myeloma has been well established, mild renal injury by C3GN in multiple myeloma patients with high levels of light chain has not been reported. Case presentation A 55-year-old Chinese man presented with proteinuria. Combined with immunofixation electrophoresis, bone marrow biopsy, and renal biopsy, he was diagnosed with IgA-type multiple myeloma accompanied by C3GN and light chain proximal tubulopathy without crystal deposits. Although he had a higher level of lambda serum-free light chain, the renal injury in this patient was mild. After treatment with four courses of BD, one course of PAD, and autologous stem cell transplantation, he achieved a very good partial hematologic response with stable renal function. Conclusions In multiple myeloma, the light chain reaches a certain level and persists, resulting in C3GN renal impairment. Early diagnosis and early intensive treatment are critical for the prognosis of such patients.

scholarly journals Multiple myeloma with crystal-storing histiocytosis, crystalline podocytopathy, and light chain proximal tubulopathy, revealed by retinal abnormalities

Medicine ◽  
2018 ◽  
Vol 97 (52) ◽  
pp. e13638 ◽  
Author(s):  
Idris Boudhabhay ◽  
Chérif Titah ◽  
Alexis Talbot ◽  
Stéphanie Harel ◽  
Jérôme Verine ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy

scholarly journals A Case of Fanconi Syndrome due to Light Chain Proximal Tubulopathy

2017 ◽  
Vol 106 (4) ◽  
pp. 813-819 ◽  
Author(s):  
Mika Yamashige ◽  
Shunsuke Goto ◽  
Junko Nakano ◽  
Kimihiko Goto ◽  
Kentaro Nakai ◽  
...  
Keyword(s):  
Light Chain ◽  
Fanconi Syndrome ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy
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