Nephrotic syndrome and acute renal failure in non-Hodgkin lymphoplasmacytic lymphoma

Abstract

Two patients with lymphoplasmacytic lymphoma, and monoclonal proteins of IgM in one, and IgG and lambda light chains in the second patient, nephrotic syndrome and acute renal failure are reported. A 58-year-old man previously treated for pre-B acute lymphoblastic leukemia, developed 3 years later nephrotic syndrome as a complication of lymphoplasmacytic lymphoma and high-paraprotein IgM kappa type. Immunofluorescent analysis of kidney biopsy showed extensive IgM and light kappa chain deposits, which caused membranoproliferative glomerulonephritis. Treatment with cyclophosphamide was ineffective and patient died 2 months later. The second patient is a 42-year-old female diagnosed with lymphoplasmacytic lymphoma and paraprotein IgG lambda type. The course of the disease was fulminant with developing nephrotic syndrome and fatal acute renal failure. Immunofluorescent and light microscopic studies of kidney biopsy showed signs of immunotactoid glomerulonephritis with deposits of IgG and C3. Hemodyalises and cytostatic therapy were without response and she died after 45 days.

Membranoproliferative glomerulonephritis complicating Waldenström’s macroglobulinemia

“…Thus, the finding of the glomerulonephritis was ‘incidental’ in the context of a renal biopsy performed for acute renal failure. Moreover, proliferative GN reported in the context of monoclonal lymphoproliferations is often associated with nephrotic syndrome [1,8,9], which was not true for our case, so that the MPGN pattern of injury was even less expected. Secondly, from the morphological point of view our case was reminiscent of CG-GN with monoclonal type I CG [10,11], which would go very well in line with the glomerular proliferation and the purpuric skin manifestation.…”

“…However, several reports in literature can be found describing monoclonal intrarenal IgM deposits and marked glomerular proliferation sometimes with the typical picture of MPGN without proof of CG [1,4,8,13-15]. Interestingly, negativity for CG is also present in a recently described proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) [16].…”

Bilateral ocular involvement as a presentation of Waldenström’s macroglobulinemia

“…Osseous and renal lesions are rare [2] Hyperviscosity retinopathy is the most commonly reported ocular abnormality in Waldenström's macroglobulinemia…”