Nephrotic syndrome associated with a clonal T-cell leukemia of large granular lymphocytes with cytotoxic function.
A 51-year-old man presented with a T-cell leukemia of large granular lymphocytes and rapidly developed a nephrotic syndrome due to presumptive minimal-change glomerulopathy. The E-rosette+, Ia+ cells demonstrated cytotoxic activity similar to that of natural killer lymphocytes but lacked other T-subset markers, except that one third of them bore Fc(IgG) receptors. Cytogenetic analysis revealed loss of chromosome 10 and the translocation (1;10)(p11;q11) in all metaphases. Regression of the leukemia after chemotherapy was accompanied by a dramatic resolution of the nephrotic syndrome, suggesting that the activated granular lymphocytes induced the renal lesion. The close association of a clonal T-lymphoproliferative disorder with minimal-change nephrotic syndrome lends further support to current views implicating activated T cells or their products in the pathogenesis of this glomerulopathy.
D002900:Chromosomes, Human, 1-3; D002906:Chromosomes, Human, 6-12 and X; D006801:Humans; D007938:Leukemia / Q000150:complications* / Q000235:genetics / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009402:Nephrosis, Lipoid / Q000209:etiology* / Q000276:immunology; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology / Q000473:pathology*; D014178:Translocation, Genetic