light chain deposition disease


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light chain disease

A nephropathy which presents with nephrotic range proteinuria and rapidly progressive renal failure due to deposition of electron-dense material within tubular and glomerular basement membranes.

Clinical findings
Nephrotic syndrome, heart failure, arrhythmias, liver disease, anorexia, nausea, weight loss, anaemia, increased creatinine, and neurologic disease; similar clinical findings occur in heavy chain deposition.

light chain deposition disease

Bence-Jones myeloma, light chain disease Oncology A paraproteinemia linked to renal amyloidosis, which occurs in 20% of Pts with immunoproliferative disorders; both are characterized by deposits of fibrillar and nonfibrillar monoclonal light chains in various tissues Clinical Rapidly progressive renal failure due to renal tubular blockage by Bence-Jones proteins, nephrotic syndrome, heart failure, arrhythmias, liver disease, neurologic disease; similar clinical findings are associated with heavy chain deposition
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References in periodicals archive ?
Systemic light chain deposition disease presenting as multiple pulmonary nodules: a case report and review of the literature.
Nodular pulmonary light chain deposition disease: an entity associated with Sjogren syndrome or marginal zone lymphoma.
Light chain deposition disease is histologically indistinguishable from nodular pulmonary amyloidosis; however, Congo red staining is negative (not shown) (hematoxylin-eosin, original magnifications x20 [A] and x400 [B]).
Light chain deposition disease of the liver associated with AL-type amyloidosis and severe cholestasis.
Light chain deposition disease presenting with hepatomegaly: an association with amyloid-like fibrils.
Tichy et al., "Induction treatment of light chain deposition disease with bortezomib: rapid hematological response with persistence of renal involvement," Leukemia and Lymphoma, vol.
This pattern of interstitial disease could be conceptualized as an interstitial form of light chain deposition disease without manifestations in other renal compartments.
Among the glomerular expressions, "minimal" change, mesangioproliferative (Figure 8), membranoproliferative, and crescentic patterns have been documented in light chain deposition disease. (5,10,12,46) These morphologic patterns are easily conceptualized as occurring as a result of the interplay of 2 growth factors: platelet-derived growth factor [beta], promoting mesangial cell proliferation, and transforming growth factor [beta], responsible for matrix deposition.
In early cases of light chain deposition disease, when the glomeruli appear essentially unremarkable by light microscopy and fluorescence for monoclonal light chains is inconclusive, lightchain deposits may not be recognizable ultrastructurally.
Metachronous development of nonamyloidotic lambda light chain deposition disease and IgG heavy chain amyloidosis in the same patient.
[1] Nonstandard abbreviations: FLC, free light chain; NSMM, nonsecretory multiple myeloma; AL, primary systemic amyloidosis; LCDD, light chain deposition disease; LCMM, light chain multiple myeloma; IFE, immunofixation electrophoresis; PCD, plasma cell disorder; MCUS, monoclonal gammopathy of undetermined significance; and SMM, smoldering multiple myeloma.
We used urine samples obtained from inpatients with multiple myeloma (n = 9), benign monoclonal gammopathy (n = 1), primary macroglobulinemia (n = 1), light chain deposition disease (n = 1), and primary amyloidosis (n = 5).