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Blood, 15 October 2008, Vol. 112, No. 8, pp. 3082-3087.
Prepublished online as a Blood First Edition Paper on July 22, 2008; DOI 10.1182/blood-2008-05-154609.
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Submitted May 1, 2008
Accepted June 29, 2008
Long term outcomes of patients with advanced-stage cutaneous T cell lymphoma and large cell transformation
Suzanne O Arulogun, H. Miles Prince*, Jonathan Ng, Stephen Lade, Gail F. Ryan, Odette Blewitt, and Christopher McCormack
Department of Haematology and Medical Oncology, Peter MacCallum Cancer Centre, East Melbourne, Australia
Monash University, Clayton, Melbourne, Australia
Department of Dermatology, St Vincent's Hospital, Melbourne, Australia
Department of Pathology, Peter MacCallum Cancer Centre, East Melbourne, Australia
Division of Radiation Oncology, Peter MacCallum Cancer Centre, East Melbourne, Australia
University of Melbourne, Parkville, Melbourne, Australia
* Corresponding author; email: miles.prince{at}petermac.org.
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 with early-stage disease, compared with stage IIB (27%) and stage IV (56%-67%) disease. The median OS from diagnosis of LCT was two years. We confirm that the incidence of LCT is strongly dependent on tumor stage at diagnosis and demonstrate a much lower overall risk of LCT than previously reported.

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