Nonerythrodermic, leukemic variant of cutaneous T-cell lymphoma with indolent clinical course: Th2-type tumor cells lacking T-cell receptor/CD3 expression and coinfiltrating tumoricidal CD8+T cells

As typically represented by Sezary syndrome, the leukemic Form of cutaneous T-cell lymphoma (CTCL) mostly exhibits erythroderma. A patient with CTCL h ad slowly developing skin tumors as well as chronic leukemia. The tumor cel l was CD4(+)CD7(-) Th2 cells lacking T-cell receptor/CD3 complex and persis tently occupied 27% to 48% of peripheral blood lymphocytes. In skin tumors, only 13% of tumor-infiltrating lymphocytes were malignant cells and substa ntial numbers of nonmalignant CD4(+) or CD8(+) T cells and B cells coinfilt rated. CD8(+)-infiltrating T cells had cytotoxic activity against the malig nant T cell. Our case demonstrates the existence of the leukemic form of CT CL presenting with skin manifestation other than erythroderma and parapsori atic patches. The nonerythrodermic feature and indolent course may be assoc iated with the lack of T-cell receptor/CD3 expression and coinfiltration of a high percentage of nontumor lymphocytes, including tumoricidal CD8(+) T cells.