Multiple myeloma (MM) is consistently preceded by precursor states of monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM). These represent a continuum of progression of the tumor burden from the absence of symptoms or signs of end-organ damage towards full-blown symptomatic disease. MGUS, by definition presenting by monoclonal gammopathy without end organ damage, in fact might be associated with the numerous end organ lesions, first of all pathologic renal conditions. The term monoclonal gammopathy of renal significance (MGRS) was proposed by International Kidney and Monoclonal Gammopathy Research Group in order to discriminate the pathologic nature of these diseases from the truly benign MGUS. Spectrum of MGRS, caused by the deposition of monoclonal immunoglobulin's or fragments thereof as organized and non-organized deposits, includes more commonly AL amyloidosis, monoclonal immunoglobulin deposition disease (MIDD) and light-chain proximal tubulopathy (LCPT). Same variants are seen in patients with MM and SMM. We present a rare case of combined immunoglobulin G kappa nephropathy: MIDD and proximal tubulopathy in a patient, manifested with acute kidney injury and diagnosed with SMM after 6 years of scrutinous repeated evaluation. .
BackgroundMultiple myeloma (MM) is a plasma-cell dyscrasia presenting with generalized neoplastic changes in bones, accompanied by impaired haematopoiesis and susceptibility to infections. The diagnosis is based on histologic, serologic, and radiographic features: bone marrow clonal plasma cells; monoclonal protein in the serum or urine; and end-organ damage, evidenced by renal impairment, hypercalcemia, anemia, or lytic bone lesions. Monoclonal gammopathy of undermined significance (MGUS) is a pre-malignant disorder, characterized by presence of monoclonal gammopathy without end organ damage. MGUS tend to progress over time to MM, lymphoproliferative disorders and AL amyloidosis with the rate about 1% per year. Asymptomatic or smoldering multiple myeloma (SMM) is a heterogeneous clinical entity where a subset of patients has an indolent course that mimics MGUS, whereas others have a more aggressive course that has been described as "early myeloma". MM is consistently preceded by precursor states of MGUS and SMM, these represent a continuum of progression of the tumor burden from the absence of symptoms or signs of end-organ damage towards full-blown symptomatic disease [1][2][3][4][5][6][7][8].Renal damage in MM is mainly caused by the deposition of monoclonal immunoglobulin's (Ig) or fragments thereof as organized (casts, crystals, fibrils, microtubules) and non-organized deposits, involving all compartments of the renal parenchyma: glomeruli,