Light-chain Deposition Disease of the Kidney: A Case Report

ArticleinUltrastructural Pathology 36(2):134-8 · April 2012with12 Reads
DOI: 10.3109/01913123.2011.642464 · Source: PubMed
A 41-year-old man was admitted for evaluation of nephrotic syndrome associated with microhematuria, hypertension, and moderate renal failure. In serum and urine samples, monoclonal IgG-lambda was detected. Bone marrow examination showed normal representation of all cell lines with normal range of plasma cells. Renal biopsy demonstrated diabetes-like nodular glomerulosclerosis. Immunofluorescence failed to demonstrate the presence of kappa or lambda light chains in the kidney. Electron microcopy showed granular electron-dense deposits along the glomerular basement membranes and in the mesangial nodules. The patient was diagnosed as having light-chain deposition disease (LCDD) without evidence of plasma cell dyscrasia. This report was designed to stress the significant challenges that remain in the diagnosis of LCDD-related glomerulopathy. The salient morphological features that help in making an accurate diagnosis are discussed.
  • [Show abstract] [Hide abstract] ABSTRACT: Light-, light- and heavy-, and heavy-chain deposition diseases belong to a family of diseases that include light-chain (AL)-amyloid, nonamyloid fibrillary and immunotactoid glomerulonephritis, and cryoglobulinemic glomerulonephritis, in which monoclonal Ig or their subunits become deposited in kidney. In clinical and pathologic terms, light-, light- and heavy-, and heavy-chain deposition diseases essentially are similar and are characterized by prominent renal involvement with severe renal failure; extrarenal manifestations; diabetes-like nodular glomerulosclerosis; marked thickening of tubular basement membranes; and monotypic deposits of light chain, mostly kappa, and/or heavy chain that feature a nonorganized granular, electron-dense appearance by electron microscopy. The most common cause is myeloma. Recent progress has been made in the understanding of the molecular pathomechanisms of Ig-chain deposition and extracellular matrix accumulation, which opens up new therapeutic avenues in addition to eradication of the Ig-secreting plasma cell clone. Because these diseases represent a model of glomerular and interstitial fibrosis that is induced by a single molecule species, a better understanding of their pathomechanisms may help to unravel the pathophysiology of kidney fibrosis and renal disease progression.
    Full-text · Article · Dec 2006
  • [Show abstract] [Hide abstract] ABSTRACT: This review is based on our own observations of 14 patients with LCDD at Necker Hospital between 1973 and 1982 and on the data in the literature. Several of our case reports have already been published, together with a series from Tenon Hospital in Paris. In plasma cell dyscrasias (including myeloma, LCDD, and AL amyloidosis), the clinical features depend critically on the substances released by the 'dysregulated' plasma cell clone: for example, osteoclast activating factor in some myeloma patients with severe bone involvement; potentially nephrotoxic monoclonal light chains in Bence Jones myeloma leading to the classical myeloma kidney; abnormal light chains prone to tissue deposition in LCDD or light chains highly susceptible to proteolysis in macrophage lysosomes in AL amyloidosis. Further investigations are needed to clarify the cell defects leading to such a synthesis, as well as the intermediary mechanisms involved.
    Article · Aug 1984
  • [Show abstract] [Hide abstract] ABSTRACT: The renal biopsies from 47 patients with plasma cell dyscrasias were studied by light and electronmicroscopy, and by immunohistochemical methods. This report is primarily concerned with the ultrastructural features of 24 cases of Bence Jones cast nephropathy and of ten cases of light chain deposit disease. In Bence Jones cast nephropathy, crystals derived from light chain proteins were detected in the majority of cases within the casts or in tubular cells and appeared to be related to the "hard" and "fractured" appearance of the casts as well as to the presence of foreign body type giant cells, the latter probably being of monocyte-macrophage origin. In light chain deposit disease, linear deposits of light chain proteins (eight kappa and two lambda) were present in a subendothelial position along the glomerular basement membrane and along the outer aspect of the tubular basement membranes in all cases, quite often in the mesangial matrix, but much less commonly in the interstitium and in the wall of small arteries. The light and electronmicroscopic features of both Bence Jones cast nephropathy and light chain deposit disease can be considered diagnostic for plasma cell dyscrasia. The possible pathogenetic mechanisms of these two different forms of renal involvement are discussed briefly.
    Article · Oct 1987
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