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Cuadernos Hospital de Clínicas
Print version ISSN 1562-6776
Abstract
CALANI CUSI, Fanny and HUANCA MAMANI, Irsen. Kidney involvement in idiopathic light chain disease. Report of one case. Cuad. - Hosp. Clín. [online]. 2017, vol.58, n.2, pp.35-40. ISSN 1562-6776.
Multiple myeloma (MM) is the monoclonal gammopathy (MG) that most frequently compromises renal function; however, a growing number of renal diseases associated with monoclonal gammopathies are being recognized. Monoclonal gammopathies (MG) are a set of entities characterized by the abnormal production and secretion into the blood of a monoclonal immunoglobulin (Ig) from a single clone of plasma cells or a fragment thereof (heavy, light chains orboth) which can be deposited in the organs in an organized way such as crystals, fibrils or microtubules, orin an unorganized (granular) form. This Ig is mainly deposited in the kidney, not only because it is a very vascularized organ, but also because the renal tubule plays a predominant role in the metabolism of Igs. The diagnosis ofrenal involvement has undergone changes in the last decades, being more and more certain due to the development and routine implementation of different laboratory techniques (stains with specific antibodies against kappa and lambda light chains, study with electron microscopy (EM), development of increasingly sensitive techniques for detecting the monoclonal component in blood or urine). Renal involvement in these pathologies is often associated with malignant MG, generally associated with light chain deposits. Increasingly, there are cases of renal dysfunction associated with MG, and progression to terminal renal disease and high rates of recurrence after renal transplantation are evident in many of these.
Keywords : light chain deposition disease; monoclonal immunoglobulin deposition disease; monoclonal gammopathy; proteinuria; nephrotic syndrome.