We describe a 57-year-old woman who presented with a constitutional sy
ndrome, glomerulonephritis, and lymphocytosis, The phenotypic study, u
sing flow cytometry, showed an expansion of natural killer (NK) cells
(CD2+, CD3-, CD16+, CD56+, and CD7+), We performed a functional study
of peripheral blood mononuclear cells (PBMCs) and of purified CD16+ ce
lls (NK cells) and CD3+ cells (normal T cells), The expanded NK cell p
opulation, CD16+, did not proliferate with phytohemagglutinin (PHA) or
anti-CD3 but showed a dose-dependent proliferation with recombinant i
nterleukin-2 (rIL-2) and also proliferated with phorbol dibutyrate, Th
is population showed very strong NK and lymphokine-activated killer ce
ll (LAK) activities, The patient's symptoms resolved spontaneously wit
hout treatment, Three years later, however, there is still abnormal re
nal function, and the expansion of NK cells persists, although with no
indication of malignancy, We review the features of the different lar
ge granular lymphocyte proliferations and their seldom described relat
ionship with renal disease, (C) loss Wiley-Liss, Inc.