Gilboa N, Durante D, Guggenheim S, Lacher J, Holman R, Schorr W, Garfield D, McIntosh R M
Nephron. 1979;24(5):223-31. doi: 10.1159/000181721.
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.
2例患者在诊断为慢性淋巴细胞白血病数年之后出现肾病综合征。在这两例患者中,光镜检查均显示为膜增生性肾小球肾炎。电镜检查和免疫荧光染色发现电子致密沉积物以及免疫球蛋白和C3的沉积。两名患者均有单一组分IgG冷球蛋白血症。洗脱的肾小球结合蛋白仅含IgG。患者血清、冷球蛋白和肾脏洗脱液中的IgG仅含κ轻链。通过Clq结合试验在血清和冷球蛋白中检测到免疫复合物。免疫吸附研究显示患者血清、冷球蛋白和肾脏洗脱液中存在抗IgG抗体。使用患者血清、冷球蛋白和肾脏洗脱液对患者肾脏冰冻切片进行的直接免疫荧光研究呈阳性,为肾小球肾炎的免疫复合物性质提供了额外证据。我们患者的免疫组化研究提示存在循环IgG-抗IgG免疫复合物,并且它们可能参与了这2例患者肾小球肾炎和肾病综合征的发病机制。