Abstract
AbstractMonoclonal gammopathy of renal significance (MGRS) refers to a small B-cell clone associated with renal disease induced by the secreted monoclonal Ig (MIg). Renal lesions, independent of the tumor burden, are governed by the MIg characteristics and involve either direct (deposition) or indirect mechanisms (autoantibody activity, complement activation). The spectrum of MGRS encompasses glomerular and tubulo-interstitial lesions classified into 3 categories according to the composition and ultra-structural appearance of deposits. The most frequent is AL amyloidosis, defined by organized fibrillar deposits of monoclonal light chains (LC). The diagnosis of each specific nephropathy, suggested by the analysis of renal (composition of proteinuria) and extra-renal manifestations, is confirmed in most cases by light, immunofluorescence and electron microscopy studies of a kidney biopsy. Early diagnosis and rapid achievement of a deep haematological response through clone-targeted chemotherapy determine the outcomes. A detailed hematologic workup is warranted to characterize the MIg (serum and urine electrophoresis and immunofixation, serum free LC) and to identify the underlying clone (bone marrow flow cytometry, cytogenetics).This review focuses on the recent advances in the diagnosis of MGRS, highlighting the value of novel techniques (molecular biology, proteomics) which should facilitate the management of these rare and complex disorders.
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