Abstract
Severe nephrotic syndrome developed suddenly in a 67-year-old man. IgM M-component and bone marrow findings indicated a diagnosis of Waldenström's macroglobulinaemia. High-titre IgM glomerular autoantibodies were found to reside mainly in the M-component. Immunofluorescent (IF) studies on serial kidney biopsies showed extensive IgM deposits that disappeared after treatment. Light microscopy of kidney biopsy appeared only slightly altered, but combined with electron microscopy could demonstrate changes that correlated well with IF findings. The latest biopsy showed interstitial infiltration in the kidney of atypical lympho-histiocytic cells. Morphological and immunological examination indicated that pathogenetic events started with minimal-change glomerulonephritis, causing release of glomerular antigen, that finally triggered a monoclonal IgM response and lymphoproliferative reaction. Intermittent treatment with chlorambucil and corticoids completely abolished the nephrotic syndrome and at the same time the changes in renal morphology largely disappeared.
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- COONS A. H., KAPLAN M. H. Localization of antigen in tissue cells; improvements in a method for the detection of antigen by means of fluorescent antibody. J Exp Med. 1950 Jan 1;91(1):1–13. doi: 10.1084/jem.91.1.1. [DOI] [PMC free article] [PubMed] [Google Scholar]
- DIXON F. J., FELDMAN J. D., VAZQUEZ J. J. Experimental glomerulonephritis. The pathogenesis of a laboratory model resembling the spectrum of human glomerulonephritis. J Exp Med. 1961 May 1;113:899–920. doi: 10.1084/jem.113.5.899. [DOI] [PMC free article] [PubMed] [Google Scholar]
- DUNCAN D. A., DRUMMOND K. N., MICHAEL A. F., VERNIER R. L. PULMONARY HEMORRHAGE AND GLOMERULONEPHRITIS. REPORT OS SIX CASES AND STUDY OF THE RENAL LESION BY THE FLUORESCENT ANTIBODY TECHNIQUE AND ELECTRON MICROSCOPY. Ann Intern Med. 1965 May;62:920–938. doi: 10.7326/0003-4819-62-5-920. [DOI] [PubMed] [Google Scholar]
- Idelson B. A., Smithline N., Smith G. W., Harrington J. T. Prognosis in steroid-treated idiopathic nephrotic syndrome in adults. Analysis of major predictive factors after ten-year follow-up. Arch Intern Med. 1977 Jul;137(7):891–896. [PubMed] [Google Scholar]
- Kijlstra A., Van Der Lelij A., Knutson W., Fleuren G. J., Vanes L. A. The influence of phagocyte function on glomerular localization of aggregated IgM in rats. Clin Exp Immunol. 1978 May;32(2):207–217. [PMC free article] [PubMed] [Google Scholar]
- LARCAN A., RAUBER G., STREIFF F. [Renal manifestations of Waldenstrom's disease]. J Urol Nephrol (Paris) 1962 Jan-Feb;68:57–78. [PubMed] [Google Scholar]
- Laurell C. B. Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. Anal Biochem. 1966 Apr;15(1):45–52. doi: 10.1016/0003-2697(66)90246-6. [DOI] [PubMed] [Google Scholar]
- MacKenzie M. R., Fudenberg H. H. Macroglobulinemia: an analysis for forty patients. Blood. 1972 Jun;39(6):874–889. [PubMed] [Google Scholar]
- Marquardt H., Wilson C. B., Dixon F. J. Isolation and immunological characterization of human glomerular basement membrane antigens. Kidney Int. 1973 Feb;3(2):57–65. doi: 10.1038/ki.1973.12. [DOI] [PubMed] [Google Scholar]
- Martelo O. J., Schultz D. R., Pardo V., Perez-stable E. Immunologically-mediated renal disease in Waldenström's macroglobulinemia. Am J Med. 1975 Apr;58(4):567–575. doi: 10.1016/0002-9343(75)90133-3. [DOI] [PubMed] [Google Scholar]
- Morel-Maroger L., Basch A., Danon F., Verroust P., Richet G. Pathology of the kidney in Waldenström's macroglobulinemia. Study of sixteen cases. N Engl J Med. 1970 Jul 16;283(3):123–129. doi: 10.1056/NEJM197007162830304. [DOI] [PubMed] [Google Scholar]
- Morel-Maroger L., Verroust P. Glomerular lesions in dysproteinemias. Kidney Int. 1974 Apr;5(4):249–252. doi: 10.1038/ki.1974.33. [DOI] [PubMed] [Google Scholar]