title: Primary cutaneous pleomorphic small T-cell lymphoma. A review of 11 cases. The French Study Group on Cutaneous Lymphomas.
abstract: METHODS
  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.
authors.affiliation: Department of Dermatology, Henri-Mondor Hospital, Creteil, France.
authors.forename: D
authors.initials: D
authors.lastname: Friedmann
authors.forename: J
authors.initials: J
authors.lastname: Wechsler
authors.forename: M H
authors.initials: MH
authors.lastname: Delfau
authors.forename: E
authors.initials: E
authors.lastname: Estève
authors.forename: J P
authors.initials: JP
authors.lastname: Farcet
authors.forename: A
authors.initials: A
authors.lastname: de Muret
authors.forename: A
authors.initials: A
authors.lastname: Parneix-Spake
authors.forename: L
authors.initials: L
authors.lastname: Vaillant
authors.forename: J
authors.initials: J
authors.lastname: Revuz
authors.forename: M
authors.initials: M
authors.lastname: Bagot
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000276:immunology / Q000473:pathology / Q000628:therapy; D016410:Lymphoma, T-Cell, Cutaneous / Q000276:immunology / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000473:pathology / Q000628:therapy