IgM Monoclonal Gammopathy Accompanied by Nodular Glomerulosclerosis, Urine-Concentrating Defect, and Hyporeninemic Hypoaldosteronism

Abstract

A 54-year-old male had monoclonal IgM-kappa light chains in the serum and free monoclonal kappa light chains in the urine. Renal biopsy revealed nodular glomerulosclerosis associated with the accumulation of kappa light chains. Isolated microscopic hematuria was present for over 1 year. He also showed a defect in urine concentration for which the light chains deposited along the basement membrane in the inner medullary tubules were judged to be responsible. When the glomerular filtration rate fell to 30 ml/min, the syndrome of mild renal failure, decreased potassium excretion, and hyporeninemic hypoaldosteronism developed, suggesting that kappa light chain nephropathy is a cause of this syndrome. Chemotherapy appeared to have some beneficial effects in maintaining renal function.

Onco-Nephrology

“…Subsequent case reports described immunotactoid glomerulopathy and nonamyloid fibrillary glomerulopathy (56,57), cryoglobulinemia-related GN (58), crescentic GN (59,60), light-chain deposition disease (LCDD) (61), and MPGN without cryoglobulinemia (62). The incidence of renal complications of malignant IgMsecreting proliferation has decreased, mostly because of improved treatment of WM, the major cause of those nephropathies.…”

Renal Lesions Associated with IgM-Secreting Monoclonal Proliferations

“…Renal symptoms in the three patients (11,12, and 13) with membranoproliferative glomerulonephritis (MPGN) consisted of impaired renal function in all patients associated with nephrotic syndrome in two cases (12 and 13). Urinalysis showed microscopic hematuria in all three patients.…”

“…Subsequent case reports included immunotactoid and nonamyloid fibrillary glomerulopathy (4,5), cryoglobulinemia-related glomerulonephritis (6), and crescentic glomerulonephritis (7,8). Cases of cast nephropathy (9), Fanconi syndrome (10), and LC deposition disease (11) as a result of free LC toxicity have also been reported in patients who presented with a circulating monoclonal IgM.…”

Proliferative glomerulonephritis with monoclonal IgM-κ deposits in chronic lymphocytic leukemia/small lymphocytic leukemia: case report and review of the literature

“…Although glomerulopathy secondary to monoclonal IgM deposition is rare, the most characteristic lesion is intracapillary deposits of IgM with or without cryoglobulinemia and AL amyloidosis [15,16]. Otherwise, immunotactoid/fibrillary glomerulopathy, cryoglobulinemia-related glomerulopathy, and light chain deposition disease were also reported [10,[17][18][19]. Furthermore, we could identify 19 cases suitable for the concept of PGNMIMD in the literature [10,14,15,[20][21][22][23].…”