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A Newly Described, Rare Variant of Cutaneous T-Cell Lymphoma

The vast majority of cutaneous T-cell lymphomas are CD4+ lymphomas with clonal rearrangements of the ß T-cell receptor. This reflects the fact that 95 percent of mature T cells express the ß heterodimer of the T-cell receptor. Rare cases of cutaneous T-cell lymphomas that express the T-cell receptor (present on about 5 percent of normal T cells) have been reported. In this study, the authors examined a series of 3 cases of cutaneous T-cell lymphoma to characterize more fully the clinical, histologic, and immunologic features of this unusual lymphoma.

All 3 patients were men, ranging in age from 46 to 74, who presented with multiple cutaneous plaques that clinically resembled mycosis fungoides, tumors, or subcutaneous nodules. Lymphadenopathy and hepatosplenomegaly were not present in any of the patients. Histologic features demonstrated a range of changes, including a dense, bandlike lymphocytic infiltrate with marked epidermotropism; Pautrier's microabscesses; and involvement of the subcutis. The lymphocytes were cytologically abnormal but did not demonstrate the usual cerebriform pattern seen in mycosis fungoides. Nucleoli were occasionally present, as were mitoses. All cases demonstrated populations of CD3+ T cells. Two of the cases did not express either CD4 or CD8, and the third expressed only CD8. The cells also expressed TIA-1, perforin, and granzyme B, as is characteristic of cytotoxic T cells. Clonal rearrangements were detected by polymerase chain reaction. All patients experienced aggressive clinical courses that were resistant to multiagent chemotherapy.

Comment: It is important to recognize this rare yet important subtype of cutaneous T-cell lymphoma. Despite its clinical and histologic similarity to mycosis fungoides, cutaneous T-cell lymphoma apparently has a very different clinical course and prognosis. The number of cases in this study is small, thus conclusions must be drawn with caution. Regardless, the results suggest that it would be prudent to evaluate fully -- by means of immunophenotyping or T-cell gene rearrangement studies -- the nature of malignant cells in patients with cutaneous T-cell lymphoma.

— BR Smoller

Published in Journal Watch Dermatology September 11, 2000

Citation(s):

Toro JR et al. T-cell lymphoma of the skin. Arch Dermatol 2000 Aug 136 1024-1032.

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