Prognostic Model for Survival in Advanced-Stage Mycosis Fungoides and Sézary Syndrome


Key Points

  • Stage IV disease, age > 60 years, large cell transformation in the skin, and elevated serum LDH levels were independent prognostic factors for worse survival in patients with advanced-stage mycosis fungoides and Sézary syndrome.
  • Use of these risk factors in a prognostic model distinguished low-, intermediate-, and high-risk groups with significantly different survival rates.

As reported by Scarisbrick et al in the Journal of Clinical Oncology, a Cutaneous Lymphoma International Consortium study in a large population of patients with advanced-stage mycosis fungoides and Sézary syndrome has identified independent prognostic factors for overall survival that can be used in a prognostic model to distinguish risk groups across clinical stages.

Study Details

The study included staging data from 1,275 patients with advanced mycosis fungoides/Sézary syndrome from 29 international sites. Ten candidate markers for survival were identified from literature review, consisting of stage, age, sex, cutaneous histologic features of folliculotropism, CD30 positivity, proliferation index, large cell transformation, white blood cell count/lymphocyte count, serum lactate dehydrogenase (LDH) level, and identical T-cell clone in blood and skin.

Survival by Clinical Stage

Median overall survival among all patients was 63 months, with 2- and 5-year survival rates of 77% and 52%. Median overall survival was 68 months among patients with stage IIB disease, not reached in those with stage III disease, 48 months in those with stage IVA disease, and 33 months among those with stage IVB disease. Predicted 5-year overall survival was 57.4% for those with stage IIB disease, 58.2% for those with stage III disease (no significant difference vs stage IIB disease), 42.9% for those with stage IVA disease (P = .003 vs IIB), and 39% for those with stage IVB disease (P = .008 vs stage IIB disease).

Prognostic Model

Of the 10 variables tested, stage IV disease (P = .009), age > 60 years (P < .001), large cell transformation in the skin (P < .001), and elevated serum LDH level (P < .001) were independent prognostic markers for worse survival on multivariate analysis. Use of these four factors in a prognostic index identified risk groups across clinical stages with significantly different 5-year overall survival rates: low risk (no or one risk factor) = 68%; intermediate risk (two risk factors) = 44% (hazard ratio [HR] = 2.09, P < .001, vs low risk); and high risk (three or four risk factors) = 28% (HR = 2.91, P < .001, vs low risk).

The investigators concluded: “To our knowledge, this study includes the largest cohort of patients with advanced-stage mycosis fungoides/Sézary syndrome and identifies markers with independent prognostic value, which, used together in a prognostic index, may be useful to stratify advanced-stage patients.”

Julia J. Scarisbrick, MBChBhons, FRCP, MD, of the University Hospital Birmingham, United Kingdom, is the corresponding author of the Journal of Clinical Oncology article.

The study was supported by the Drs. Martin and Dorothy Spatz Charitable Foundation.

The content in this post has not been reviewed by the American Society of Clinical Oncology, Inc. (ASCO®) and does not necessarily reflect the ideas and opinions of ASCO®.