Adult Fanconi syndrome progressing to multiple myeloma.

Fanconi’s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium

“…Crystalline inclusion bodies were observed in the bone marrow and plasma cells [5,9,11]. In our patient, no electron-microscopic studies could be performed, but the immunoenzymatic studies showed the presence of kappa light chains in all myelomatous cells.…”

“…By the immunocytochemical technique, some reports demonstrated the presence of kappa [11] or lambda [12] light-chain pro teins in these crystals. These crystals were also reported in histiocytes and plasma cells in the bone marrow [5,9,10]. We report a case of Fanconi's syndrome associated with kappa light-chain multiple myeloma which shows the presence of kappa light-chain deposits in the bone marrow.…”

“…Adult Fanconi's syndrome characterized by glycos uria, aminoaciduria and phosphaturia, which are mani festations of an acquired absorptive defect of proximal renal tubules, is a recognized complication of multiple myeloma [ 1 -7], Pathologic renal changes in cases of Fan coni's syndrome associated with multiple myeloma have been reported to show the presence of cytoplasmic crys tals and round or rod-like electron opaque structures [5,[8][9][10][11]. These structures are probably lysosomal inclu sions composed of altered light chains.…”

Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma

“…Literature on this identity consists of generally isolated case reports and small case series. [1][2][3][4][5][6][7][8][9] The largest series reported so far included 11 patients. 10 To better characterize the clinical presentation of these patients, we studied all 32 patients who were evaluated at Mayo Clinic (Rochester, MN) over a 34-year period.…”