Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation.

Abstract:

:Although mycosis fungoides (MF) is typically an indolent disease, patients with advanced-stage disease (stages IIB-IVB), including Sézary syndrome (SS), often have a poor outcome. A 31-year, retrospective analysis of our cutaneous lymphoma database, of 297 patients with MF and 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 5 years with a 10-year predicted OS of 32%. Age at initial diagnosis (P = .01), tumor stage (P = .01), and clinical stage (P = .001) 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 = .25). 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 2 years. We confirm that the incidence of LCT is strongly dependent on tumor stage at diagnosis, and we demonstrate a much lower overall risk of LCT than previously reported.

journal_name

Blood

journal_title

Blood

authors

Arulogun SO,Prince HM,Ng J,Lade S,Ryan GF,Blewitt O,McCormack C

doi

10.1182/blood-2008-05-154609

subject

Has Abstract

pub_date

2008-10-15 00:00:00

pages

3082-7

issue

8

eissn

0006-4971

issn

1528-0020

pii

blood-2008-05-154609

journal_volume

112

pub_type

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