Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

Nita Sally Agar; Emma Wedgeworth; Siobhan Crichton; Tracey J. Mitchell; Michael Cox; Silvia Ferreira; Alistair Robson; Eduardo Calonje; Catherine M. Stefanato; Elizabeth Mary Wain; Bridget Wilkins; Paul Fields; Alan Dean; Katherine Webb; Julia Scarisbrick; Stephen Morris; Sean J. Whittaker

doi:10.1200/JCO.2009.27.7665

Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

Nita Sally Agar, Emma Wedgeworth, Siobhan Crichton, Tracey J. Mitchell, Michael Cox, Silvia Ferreira, Alistair Robson, Eduardo Calonje, Catherine M. Stefanato, Elizabeth Mary Wain, Bridget Wilkins, Paul Fields, Alan Dean, Katherine Webb, Julia Scarisbrick, Stephen Morris, Sean J. Whittaker

Research output: Contribution to journal › Article › peer-review

688 Citations (Scopus)

Abstract

Purpose: We have analyzed the outcome of mycosis fungoides (MF) and Sezary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal. Patients and Methods: Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models. Results: The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sezary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival. Conclusion: This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

Original language	English
Pages (from-to)	4730 - 4739
Number of pages	10
Journal	Journal of Clinical Oncology
Volume	28
Issue number	31
DOIs	https://doi.org/10.1200/JCO.2009.27.7665
Publication status	Published - 1 Nov 2010

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1200/JCO.2009.27.7665

Fingerprint

Dive into the research topics of 'Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal'. Together they form a unique fingerprint.

Cite this

Agar, N. S., Wedgeworth, E., Crichton, S., Mitchell, T. J., Cox, M., Ferreira, S., Robson, A., Calonje, E., Stefanato, C. M., Wain, E. M., Wilkins, B., Fields, P., Dean, A., Webb, K., Scarisbrick, J., Morris, S., & Whittaker, S. J. (2010). Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal. Journal of Clinical Oncology, 28(31), 4730 - 4739. https://doi.org/10.1200/JCO.2009.27.7665

@article{4ace01ca69774988a56a919afde253bd,

title = "Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal",

abstract = "Purpose: We have analyzed the outcome of mycosis fungoides (MF) and Sezary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal. Patients and Methods: Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models. Results: The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sezary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival. Conclusion: This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.",

author = "Agar, {Nita Sally} and Emma Wedgeworth and Siobhan Crichton and Mitchell, {Tracey J.} and Michael Cox and Silvia Ferreira and Alistair Robson and Eduardo Calonje and Stefanato, {Catherine M.} and Wain, {Elizabeth Mary} and Bridget Wilkins and Paul Fields and Alan Dean and Katherine Webb and Julia Scarisbrick and Stephen Morris and Whittaker, {Sean J.}",

year = "2010",

month = nov,

day = "1",

doi = "10.1200/JCO.2009.27.7665",

language = "English",

volume = "28",

pages = "4730 -- 4739",

journal = "Journal of Clinical Oncology",

issn = "1527-7755",

publisher = "American Society of Clinical Oncology",

number = "31",

}

Agar, NS, Wedgeworth, E, Crichton, S , Mitchell, TJ, Cox, M, Ferreira, S, Robson, A, Calonje, E, Stefanato, CM, Wain, EM, Wilkins, B , Fields, P, Dean, A, Webb, K, Scarisbrick, J, Morris, S & Whittaker, SJ 2010, 'Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal', Journal of Clinical Oncology, vol. 28, no. 31, pp. 4730 - 4739. https://doi.org/10.1200/JCO.2009.27.7665

Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal. / Agar, Nita Sally; Wedgeworth, Emma; Crichton, Siobhan et al.
In: Journal of Clinical Oncology, Vol. 28, No. 31, 01.11.2010, p. 4730 - 4739.

Research output: Contribution to journal › Article › peer-review

TY - JOUR

T1 - Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome

T2 - Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

AU - Agar, Nita Sally

AU - Wedgeworth, Emma

AU - Crichton, Siobhan

AU - Mitchell, Tracey J.

AU - Cox, Michael

AU - Ferreira, Silvia

AU - Robson, Alistair

AU - Calonje, Eduardo

AU - Stefanato, Catherine M.

AU - Wain, Elizabeth Mary

AU - Wilkins, Bridget

AU - Fields, Paul

AU - Dean, Alan

AU - Webb, Katherine

AU - Scarisbrick, Julia

AU - Morris, Stephen

AU - Whittaker, Sean J.

PY - 2010/11/1

Y1 - 2010/11/1

N2 - Purpose: We have analyzed the outcome of mycosis fungoides (MF) and Sezary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal. Patients and Methods: Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models. Results: The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sezary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival. Conclusion: This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

AB - Purpose: We have analyzed the outcome of mycosis fungoides (MF) and Sezary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal. Patients and Methods: Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models. Results: The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sezary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival. Conclusion: This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

U2 - 10.1200/JCO.2009.27.7665

DO - 10.1200/JCO.2009.27.7665

M3 - Article

SN - 1527-7755

VL - 28

SP - 4730

EP - 4739

JO - Journal of Clinical Oncology

JF - Journal of Clinical Oncology

IS - 31

ER -

Agar NS, Wedgeworth E, Crichton S , Mitchell TJ, Cox M, Ferreira S et al. Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal. Journal of Clinical Oncology. 2010 Nov 1;28(31):4730 - 4739. doi: 10.1200/JCO.2009.27.7665