Background. Malignant thymoma is composed of neoplastic epithelial cel
ls and small lymphocytes. Rarely, patients also may have peripheral T-
lymphocytosis. These lymphocytes have been considered nonneoplastic be
cause of their microscopic appearance and immunophenotype, as well as
gene rearrangement studies. Methods. A 42-year-old man developed lymph
ocytosis 3 years after the completion of intensive combined chemoradio
therapy protocol for lymphocytic thymoma. These peripheral blood lymph
ocytes were evaluated phenotypically and genotypically. Results. Immun
ophenotyping established that the cells were CD3 positive, CD4 negativ
e, CD8 negative, T-cell receptor (TCR)-alpha/beta negative, and TCR-ga
mma/delta positive. Gene rearrangement studies with TCR-delta probe co
nfirmed the monoclonality of these cells. Chromosome analysis showed d
eletion of chromosome Y. The clinical course was progressive and had t
he features of malignant lymphoma. Conclusions. To the authors' knowle
dge, this is the first report of a patient with thymoma in whom monocl
onal proliferation of T-gamma/delta peripheral blood lymphocytes was c
onfirmed immunophenotypically and genotypically. These monoclonal TCR-
gamma/delta lymphocytes may belong to the malignant clone of the thymo
ma; however, the possibility that they represent an evolution of a sec
ond lymphatic malignancy cannot be excluded.