Manifestations of systemic light chain deposition

Expanding the pathologic spectrum of light chain deposition disease: a rare variant with clinical follow-up of 7 years

“…1,3,13 The histologic spectrum of light chain deposition disease can be quite variable, and ranges from minimal glomerular changes with or without glomerular deposits to membranoproliferative or mesangial proliferative glomerulonephritis. [2][3][4][5][6][7][8][9] These lesions can occur independently of nodular glomerulosclerosis but often occur concomitantly with nodular glomerulosclerosis in the same biopsy. All of these histologic subtypes can occur with or without crescents.…”

“…5 Since the first description of light chain deposition disease, over 150 cases have been documented in the English language medical literature. [2][3][4][5][6][7]10,[18][19][20][21][22][23] Despite the common occurrence of light chain deposition disease, our current case represents a unique manifestation of light chain deposition disease with histologic and ultrastructural features that resemble a massive immune-complex deposition form of glomerulonephritis, which has not been previously reported to our knowledge. Nodular accentuation of the glomerular tuft architecture was present focally in both biopsies, but this feature was overshadowed by the prominent accumulation of amorphous eosinophilic material corresponding to the prominent electron-dense deposits involving all mesangial regions and glomerular capillary loops.…”

“…The histologic features are frequently characterized by nodular glomerulosclerosis, but a wide spectrum of glomerular changes has been described which ranges from an absence of histologic changes by light microscopy, indistinguishable from minimal change disease, to variable degrees of mesangial alterations, to membranoproliferative glomerulonephritis. [2][3][4][5][6][7][8][9] Immunofluorescence microscopy or immunohistochemical localization is essential for establishing the diagnosis of light chain deposition disease, which is characterized by monoclonal deposition of either kappa or lambda light chains, but not both, in glomerular and tubular basement membranes, mesangium, interstitium, and vessel walls. Electron microscopy typically demonstrates granular electron-dense deposits, often with a confluent appearance, involving the glomerular and tubular basement membranes.…”

Systemic and rapidly progressive light-chain deposition disease initially presenting as tubulointerstitial nephritis

“…Initially, LCDD was reported to be a systemic disorder characterized by monoclonal light-chain deposition in systemic organs [15]. Patient survival varies from a few months to 10 years, and depends on extrarenal light-chain deposition, especially in the heart [2,16,17].…”

Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínico

“…Se suele encontrar en el curso de una discrasia de células plasmáticas, en otros trastornos linfoproliferativos, sin embargo, puede ocurrir en ausencia de cualquier trastorno hematológico, denominándose idiopática 1 . La ECL se encuentra en 5% de los pacientes con mieloma.…”