Immune deposit nephritis and single-component cryoglobulinemia associated with chronic lymphocytic leukemia. Evidence for a role of circulating IgG-anti-IgG immune complexes in the pathogenesis of the renal lesion.
2 patients developed the nephrotic syndrome several years after diagnoses of chronic lymphocytic leukemia. In both cases light microscopy showed membranoproliferative glomerulonephritis. Electron microscopy and immunofluorescent staining revealed electron-dense deposits and deposition of immunoglobulins and C3. Both patients had single-component IgG cryoglobulinemia. The eluted glomerular-bound protein contained IgG only. IgG in patients' sera, cryoglobulins, and kidney eluate had kappa light chains only. Immune complexes were detected in the sera and in the cryoglobulins by the Clq binding test. Immunoadsorption studies revealed anti-IgG antibodies in the patients' sera, cryoglobulin, and kidney eluate. Direct immunofluorescent studies using the patients' sera, cryoglobulins, and kidney eluate on frozen sections of patients' kidneys were positive, providing additional evidence for the immune complex nature of the glomerulonephritis. The immunohistochemical studies of our patients are suggestive of the presence of circulating IgG-anti-IgG immune complexes and their possible involvement in the pathogenesis of the glomerulonephritis and the nephrotic syndrome in these 2 cases.
D000936:Antigen-Antibody Complex; D003172:Complement C1 / Q000378:metabolism; D003176:Complement C3; D003450:Cryoglobulins; D005260:Female; D005921:Glomerulonephritis / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G; D007678:Kidney Glomerulus / Q000473:pathology; D007945:Leukemia, Lymphoid / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000276:immunology*; D010265:Paraproteinemias / Q000150:complications* / Q000276:immunology
D000936:Antigen-Antibody Complex; D003172:Complement C1; D003176:Complement C3; D003450:Cryoglobulins; D007074:Immunoglobulin G