Nephrotic Syndrome due to Immunologically Mediated Hypocomplementic Glomerulonephritis in a Patient of Waldenström’s Macroglobulinemia

Abstract

A 72-year-old man was diagnosed as having nephritic syndrome complicated by Waldenström’s macroglobulinemia (WM). A monoclonal IgM lamda protein and decreased serum complements were observed. The renal biopsy disclosed the capillary occluded by thrombi which was stained with IgG, IgA, IgM, C4, lamda light chain and slight kappa light chain in a granular pattern. Electron dense deposits were noted in the subendothelial spaces. An unusual case of WM who developed nephrotic syndrome due to immunologically mediated hypocomplementic glomerulonephritis is described.

Nephrotic syndrome and kidney failure due to immunocomplex-mediated renal damage in a patient with Waldenström's Macroglobulinemia: a case report

“…Furthermore, the evidence of hypocomplementemia correlates well with this suggested renal insult. Similar glomerular immunofluorescence characteristics have been described in only two previous reported cases however neither had renal impairment [ 15 , 16 ]. Even though the link between hematologic malignancies and glomerulonephritis has been well documented, the underlying pathogenesis in cases of WM is still unclear but it can be explained by the known intrinsic autoantibody activity of IgM [ 3 ].…”

“…As mentioned above, nephrotic syndrome is a rare manifestation of kidney involvement in patients with WM; in a recent case series study only 2 out of 7 patients with coexisting WM and kidney disease presented with nephrotic-range proteinuria [ 7 ]. When present, this syndrome is usually caused by amyloidosis [ 3 , 8 , 9 ]; other very unusual causes include cryoglobulinemia [ 10 , 11 ], minimal change disease [ 12 , 13 ], intracapillary monoclonal deposit disease [ 7 , 14 ] and immunocomplex-mediated glomerulonephritis [ 15 , 16 ].…”

Renal Lesions Associated with IgM-Secreting Monoclonal Proliferations

“…These data rather suggest abnormal physicochemical properties of the IgM. Glomerular capillaries seem to be a particularly vulnera- ble site for deposition of nephritogenic IgM because ultrafiltration further increases the protein concentration; however, intravascular deposition is not limited to glomerular capillaries because thrombi were also found in the kidney arterioles in three of our patients (6, 8, and 9), and in the skin (20). The propensity of complexed or deposited IgM to activate strongly the classical complement pathway might aggravate renal lesions (21).…”

Lipid-rich Glomerular Capillary Thrombi, of a Patient with Waldenstrom’s Macroglobulinemia

“…Although kidney involvement in WM is well documented, only approximately 80 biopsy-confirmed cases of have been published. AL amyloidosis is commonly considered as the main cause of nephrotic syndrome [2]. One report from single academic institution performed a retrospective 44 cases study of WM-related nephropathy [3].…”