Classification and therapeutic management of monoclonal gammopathies of renal significance

• Published in: La revue de medecine interne Vol. 39; no. 3; p. 161
• Main Authors: Javaugue, V, Bouteau, I, Sirac, C, Quellard, N, Diolez, J, Colombo, A, Desport, E, Ecotière, L, Goujon, J-M, Fermand, J-P, Touchard, G, Jaccard, A, Bridoux, F
• Format: Journal Article
• Language: French
• Published: France 01.03.2018
• Subjects: AL amyloidosis; Kidney failure; Immunoglobuline monoclonale; Maladie de dépôts d’immunoglobuline monoclonale; Monoclonal immunoglobulin deposition disease; Free light chains; Chaînes légères libres; Amylose AL; Insuffisance rénale; Monoclonal immunoglobulin
• ISSN: 1768-3122; 1768-3122
• DOI: 10.1016/j.revmed.2017.03.012

Two categories of renal disorders associated with monoclonal gammopathies are to be distinguished, according to the characteristics of the underlying B-cell clone. The first group of renal diseases always occurs in the setting of high tumor mass with production of large amounts of monoclonal immunoglobulins. The main complication is the so-called myeloma cast nephropathy, which almost invariably complicates high tumor mass myeloma. The second group includes all renal disorders caused by a monoclonal immunoglobulin secreted by a nonmalignant B-cell clone, and currently referred as a "monoclonal gammopathy of renal significance (MGRS)". This term was introduced to distinguish monoclonal gammopathies that are responsible for the development of kidney damage from those that are truly benign. The spectrum of renal diseases in MGRS is wide and its classification relies on the localization of renal lesions, either glomerular or tubular, and on the pattern of ultrastructural organization of immunoglobulin deposits. Physicochemical characteristics of the pathogenic monoclonal immunoglobulin are probably involved in their propensity to deposit or precipitate in the kidney, as illustrated by the high rate of recurrence of each specific type after kidney transplantation. Early diagnosis and efficient chemotherapy targeting the causal B-cell clone are mandatory to improve renal prognosis and patient survival.