Cutaneous T-cell lymphoma

Abstract

By conceptual definition primary cutaneous T-cell lymphomas (CTCL) constitute a heterogeneous group of non-Hodgkin’s lymphomas originating from skin-homing T lymphocytes. Clinically, they appear in considerable variability as cutaneous lesions, but mostly without signs of further nodal or systemic involvement and in many instances they keep confined to the skin for a indefinite time. Historically, mycosis fungoides (MF) and Sézary syndrome (SS) have been the primarily recognized forms of CTCL, and together they account for the majority of cases in this category. The clinical and pathological definition of additional subtypes of CTCL apart from MF and SS has led to an evolving classification of CTCL over the years, finally settled as the consensus classification of WHO and EORTC in 2005 (Willemze et al. 2005) which was then fully incorporated into the 2008 revised WHO lymphoma classification.