RARE VARIANTS OF CUTANEOUS T-CELL LYMPHOM A

Besides the classical forms of cutaneous T-cell lymphoma (C-CTCL), suc h as mycosis fungoides and Sezary's syndrome, unique variants may be e ncountered. The classification of these rare cutaneous T cell lymphoma s is problematic and controversial. Newer classifications of lymphoma in general, such as the Revised European-American Lymphoma (REAL) Clas sification, emphasize well-established clinico-pathological entities. It seems appropriate to attempt to bring greater clarity to the classi fication of cutaneous T-cell lymphomas using the same principles. In t his review, we list and characterize the rare variants of cutaneous T- cell lymphoma, such as (1) clinical, histological and immunological va riants of mycosis fungoides; (2) progressive cutaneous T-cell lymphoma (P-CTCL) including transformed classical cutaneous T-cell lymphoma (T C-CTCL) and primary progressive cutaneous T-cell lymphoma (PP-CTCL); ( 3) angiocentric and angioimmunoblastic cutaneous T-cell lymphomas; (4) large cell anaplastic, CD30(+) T-cell lymphoma; (5) HTLV-I-associated adult T-cell leukemia/lymphoma (ATLL); (6) manifestations of primary extracutaneous T-cell neoplasias; (7) unclassifiable cutaneous T-cell lymphoma.