Related Articles Long term outcomes of patients with advanced-stage cutaneous T cell lymphoma and large cell transformation. Blood. 2008 Jul 22; Authors: Arulogun SO, Prince HM, Ng J, Lade S, Ryan GF, Blewitt O, McCormack C Although mycosis fungoides is typically an indolent disease, patients with advanced-stage disease (stages IIB-IVB), including Sezary syndrome, often have a poor outcome. A 31-year, retrospective analysis of our cutaneous lymphoma database, of 297 patients with mycosis fungoides (MF) and Sezary syndrome (SS), was undertaken to study long-term outcomes and identify clinical predictors of outcome in patients with advanced-stage disease (ASD; n=92) and large cell transformation (LCT; n=22). Two-thirds of patients with ASD presented with de novo ASD. The median overall survival (OS) for ASD was five years with a 10-year predicted OS of 32%. Age at initial diagnosis (P=0.01), tumor stage (P=0.01) and clinical stage (P=0.0003) were found to be significant predictors of outcome. Patients who presented with de novo ASD demonstrated better outcomes that were not statistically significant than those with a prior diagnosis of early-stage MF (P=0.25). Large cell transformation is pathologically characterised by the morphologic change of small-/medium-sized cells to large cells that comprise >25% of a MF lesion. Transformation developed in 22 of the 297 MF/SS patients (7.4%), with a transformation rate of only 1.4% in patients w ...
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