Light Chain Deposition Disease (LCDD) is a clinical condition characterized by renal deposition of monoclonal free light chains, produced by B-cells or plasma cell clone. In LCDD, non-organized monoclonal immunoglobulin deposits along the glomerular and tubular basement membranes are composed of monoclonal light chains (kappa isotype in 92% of cases). These deposits differ from amyloidosis deposits because they do not show the typical affinity for Congo Red and do not have a fibrillar organization. We described a 64 years male patient with hypertension, proteinuria and nephrotic syndrome. Plasma cell dyscrasias diagnostic work-up evidenced only an abnormal kappa/lambda ratio and increased plasma concentrations of kappa free light chains. Serum and urine immunofixation did not demonstrated the presence on monoclonal immunoglobulin. Kidney biopsy showed a membranoproliferative glomerulonephritis pattern and renal immunofluorescence demonstrated the parietal diffuse linear staining of kappa monoclonal light chain along basement membranes. Ultrastructural appearance confirms the diagnosis of LCDD.
Il contributo della misura delle catene leggere libere plasmatiche alla diagnostica della malattia da deposito delle catene leggere
Montinaro V;
2018-01-01
Abstract
Light Chain Deposition Disease (LCDD) is a clinical condition characterized by renal deposition of monoclonal free light chains, produced by B-cells or plasma cell clone. In LCDD, non-organized monoclonal immunoglobulin deposits along the glomerular and tubular basement membranes are composed of monoclonal light chains (kappa isotype in 92% of cases). These deposits differ from amyloidosis deposits because they do not show the typical affinity for Congo Red and do not have a fibrillar organization. We described a 64 years male patient with hypertension, proteinuria and nephrotic syndrome. Plasma cell dyscrasias diagnostic work-up evidenced only an abnormal kappa/lambda ratio and increased plasma concentrations of kappa free light chains. Serum and urine immunofixation did not demonstrated the presence on monoclonal immunoglobulin. Kidney biopsy showed a membranoproliferative glomerulonephritis pattern and renal immunofluorescence demonstrated the parietal diffuse linear staining of kappa monoclonal light chain along basement membranes. Ultrastructural appearance confirms the diagnosis of LCDD.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
