Abstract
Light chain proximal tubulopathy is a rare manifestation of monoclonal gammopathy. A 73-year-old Japanese woman was noted to have urinary protein and hypertension on health examination and visited the regional clinic. She was noted to have IgG λ M protein and suspected of multiple myeloma. She was referred to us with massive proteinuria (7.5 g/g creatinine) and Bence Jones proteinuria without renal dysfunction. A renal biopsy revealed no glomerular abnormalities, but a tubular cast was observed partially in tubules without tubular atrophy or a crystalline structure. Direct Fast Scarlet staining was absent both in glomerulus and vascular wall. Immunofluorescence revealed λ light chain (LC) staining in the proximal tubules. Electron microscopy revealed nonspecific findings including increased lysosomes with irregular contours and mottled appearance. A bone marrow biopsy revealed plasma cell proliferation (35%) and multiple myeloma immunoglobulin G λ type. She showed progressive anemia and decrease of eGFR with elevated level of urinary β-2 microglobulin. She was treated with lenalidomide + dexamethasone (Ld). With Ld therapy, she achieved hematologic and nephrologic remission reducing the free LC, λ/κ ratio, urinary protein level, and urinary β-2 microglobulin level.