Pathology of the kidney in Waldenström's macroglobulinemia. Study of sixteen cases.
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Biomedical subjects
Publications and source records attributed to F Danon.
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A new type of pathological immunoglobulin was found in the serum, urine, and saliva of a young Arab patient with abdominal lymphoma and diffuse lymphoplasmacytic infiltration of the small intestine. This protein is devoid of light chains and is closely related to the alpha polypeptide chains of the gamma(A1) (Le) subclass of immunoglobulin A. It is characterized by electrophoretic heterogeneity, tendency toward polymerization, and a high carbohydrate content. No intracellular synthesis of light chain was detected.
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Monoclonal immunoglobulins (Eg) are detected in the serum of 7/29 patients treated for leukemia (6 cases) or lymphoma (1 case). All of them developped cytomegalovirus (CMV) infection. The 7 patients were children or young adults; 6/7 were in complete remission of the hematological disease. They completed chemotherapy before the onset of the viral infection and had received blood components. The monoclonal Ig are IgG (6 cases) and IgM (1 case), In 2 sera there are two M components. The level of the peak was under 1 g/100 ml (5 cases) and reached 1.5 and 1.7 g/100 ml in the 2 other sera. Polyclonal Ig are normal except in one case where there is moderate hypo-Ig; Plasmocytic infiltration was observed in none of the 7 patients. Evidence of CMV infection was confirmed by viremia and/or significant rise of complement fixing antibodies. In four cases, the monoclonal Ig disappeared in less than 6 months. The significance of the monoclonal Ig associated with CMV infection is discussed.
Anti-double stranded DNA antibodies were measured by an immunoglobulin class-specific immunoenzymatic assay (ELISA), in 450 sera from 265 patients as well as by indirect immunofluorescence using Crithidia luciliae as a substrate and, for 124 sera, by the Farr test. ELISA proved specific and reproducible and it yielded results that were well correlated with the Farr assay, with a slightly higher sensitivity of ELISA. Correlation with immunofluorescence was not as good because of the lower sensitivity of the latter method. ELISA enables the levels and isotypes of anti-DNA antibodies to be determined. Both appear to be critical parameters for a clinical interpretation of results, especially with respect to the diagnosis of systemic lupus erythematosus.
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