A case of Evans' syndrome with IgM deficiency and lymphopenia was studied b
efore and after splenectomy. The lymphopenia was as a result of profound re
duction of CD4 and CD8 cells. Study of cytokine secretion before splenectom
y revealed a spontaneous Th1- and Th2-type cytokine production, and complet
e suppression of transforming growth factor (TGF)-beta. After splenectomy,
the patient achieved clinical remission, the natural killer (NK) cell numbe
r increased and the pattern of cytokine production showed normalization of
interleukin (IL)-2, IL-4, IL-10, TGF-beta and abolition of interferon (IFN)
-gamma production. We conclude that splenectomy had a beneficial effect owi
ng to an increase in NK cells and an associated increase in TGF-beta produc
tion.