Prognostic factors in cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma (CTCL) are a heterogeneous group of neoplasms of skin-homing T cells that show considerable variation in clinical presentation, histological appearance, immunophenotype and prognosis. In recent classification schemes (WHO-EORTC; WHO 2008) roughly three categories of CTCL can be distinguished: (1) the group of classical CTCL, including mycosis fungoides (MF, variants or subtypes of MF (folliculotropic MF; pagetoid reticulosis; granulomatous slack skin) and Sézary’s syndrome (SS); (2) the group of primary cutaneous CD30-positive lymphoproliferative disorders (CD30 LPD); and (3) a group of rare often aggressive cutaneous T/NK cell lymphomas, including subcutaneous panniculitis-like T-cell lymphoma (SPTCL), extranodal NK/T-cell lymphoma, nasal type, and primary cutaneous peripheral T-cell lymphoma, not otherwise specified (PTL, NOS) as well as some rare subtypes of PTL, NOS. Classification according to these new classification schemes is the most important prognostic factor and a prerequisite for adequate management and treatment of these conditions. Herein, characteristic features of the different types of CTCL are summarized and prognostic factors within these categories are discussed.