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.

Evolution to eosinophilic leukemia with a t(5:11) translocation in a patient with idiopathic hypereosinophilic syndrome.

The idiopathic hypereosinophilic syndrome (HES) comprises a diverse group of diseases that may ultimately lead to multiorgan dysfunction and death. We present a case of a man who was followed for over 9 years with HES that underwent malignant transformation to acute leukemia with eosinophilic features. The patient's clinical acceleration was accompanied by the development of a malignant clone that was identified with banding techniques as 46,XY,t(5:11)(p15;q13). Electron microscopy reaffirmed findings reported in earlier cases of eosinophilic leukemia. At no time during his illness were cytotoxic drugs administered. In addition to delineating the natural evolution and cytostructural details of the case, we emphasize the role of cytogenetics in the predicting of malignant variants of the hypereosinophilic syndrome and in identifying eosinophilic leukemia.