PRIMARY CUTANEOUS PLEOMORPHIC SMALL T-CELL LYMPHOMA - A REVIEW OF 11 CASES

Background and Design: Cutaneous pleomorphic small T-cell lymphoma is a rare, recently recognized lymphoma, different from mycosis fungoides and Sezary syndrome. Only a few cases have been reported and no treat ment modalities have been defined. We reviewed the clinical, histologi c, immunohistochemical, molecular biologic, and follow-up data of 11 p rimary cutaneous pleomorphic small T-cell lymphomas. Results: The lesi ons presented as red purplish nodules, tumors, or plaques. The infiltr ate consisted of small pleomorphic lymphoid cells without epidermotrop ism in nine patients and with a propensity to infiltrate the dermis an d subcutaneous fat. Most cases were CD4(+)/CD8(-). A T-cell clone was detected in the skin lesions of nine patients tested. The mean follow- up was 70.1 months and the median follow-up was 20 months. Ten patient s are alive with three having persistent lesions. Interferon alfa-2a i nduced partial or complete remissions in five patients. Interferon alf a-2a combined with a regimen containing doxorubicin chlorhydrate induc ed a complete remission in a patient suffering a relapse after cycloph osphamide and interferon alone. Conclusions: Cutaneous pleomorphic sma ll T-cell lymphoma is a well-defined type of low-grade cutaneous lymph oma with favorable prognosis. Interferon and/or chemotherapy are the t reatment of choice in patients with large tumor burden.