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September 1995

Primary Cutaneous Pleomorphic Small T-Cell Lymphoma: A Review of 11 Cases

Author Affiliations

From the Departments of Dermatology (Drs Friedmann, Parneix-Spake, Revuz, and Bagot), Pathology (Dr Wechsler), and Immunology (Drs Delfau and Farcet), Henri-Mondor Hospital, Creteil, and the Departments of Dermatology (Drs Estève and Vaillant) and Pathology (Dr de Muret), Trousseau Hospital, Tours, France. A complete list of members of The French Study Group on Cutaneous Lymphomas appears on page 1014.

Arch Dermatol. 1995;131(9):1009-1015. doi:10.1001/archderm.1995.01690210039007

Background and Design:  Cutaneous pleomorphic small T-cell lymphoma is a rare, recently recognized lymphoma, different from mycosis fungoides and Sézary syndrome. Only a few cases have been reported and no treatment modalities have been defined. We reviewed the clinical, histologic, immunohistochemical, molecular biologic, and follow-up data of 11 primary cutaneous pleomorphic small T-cell lymphomas.

Results:  The lesions presented as red purplish nodules, tumors, or plaques. The infiltrate consisted of small pleomorphic lymphoid cells without epidermotropism in nine patients and with a propensity to infiltrate the dermis and 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 patients are alive with three having persistent lesions. Interferon alfa-2a induced partial or complete remissions in five patients. Interferon alfa-2a combined with a regimen containing doxorubicin chlorhydrate induced a complete remission in a patient suffering a relapse after cyclophosphamide and interferon alone.

Conclusions:  Cutaneous pleomorphic small T-cell lymphoma is a well-defined type of low-grade cutaneous lymphoma with favorable prognosis. Interferon and/or chemotherapy are the treatment of choice in patients with large tumor burden.(Arch Dermatol. 1995;131:1009-1015)