Nephrotic syndrome associated with a clonal T-cell leukemia of large granular lymphocytes with cytotoxic function

Arch Intern Med. 1986 Sep;146(9):1827-9.


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.

Publication types

  • Case Reports

MeSH terms

  • Chromosomes, Human, 1-3
  • Chromosomes, Human, 6-12 and X
  • Humans
  • Leukemia / complications*
  • Leukemia / genetics
  • Leukemia / immunology
  • Male
  • Middle Aged
  • Nephrosis, Lipoid / etiology*
  • Nephrosis, Lipoid / immunology
  • T-Lymphocytes, Cytotoxic / immunology
  • T-Lymphocytes, Cytotoxic / pathology*
  • Translocation, Genetic