A syndrome associating a polyclonal increase of B-cells with binucleat
e lymphocytes in peripheral blood and a polyclonal increase of serum I
gM, accompanied or not by splenomegaly and lymphadenopathy, has been r
eported in young to middle-aged women who are cigarette smokers. The m
ajority of patients were HLA-DR7 positive and their clinical course re
mained stable over periods of years. We report four cases of this synd
rome. In all patients, DNA analysis provided additional evidence that
the lymphocytosis was polyclonal, while in one patient who met all oth
er symptomatic criteria the total lymphocyte count was normal. This wo
uld suggest that unless careful attention is paid to the morphology of
the lymphocytes, the syndrome may go undiagnosed.