This Article  • Full text  • PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (54)  • Contact me when this article is cited  Related Content  • Related articles  • Similar articles in this journal  Topic Collections  • Dermatologic Disorders  • Prognosis/ Outcomes  • Dermatologic Disorders, Other  • Hematology/ Hematologic Malignancies  • Leukemias/ Lymphomas  • Alert me on articles by topic  Social Bookmarking   What's this?

Clinical Prognostic Factors and Risk for Disease Progression

Youn H. Kim, MD; Howard L. Liu, MD; Serena Mraz-Gernhard, MD; Anna Varghese, BA; Richard T. Hoppe, MD

Arch Dermatol. 2003;139:857-866.

Objectives  To study and update the clinical characteristics and long-term outcome of our patients with mycosis fungoides (MF) and Sézary syndrome (SS), and to identify important clinical factors predictive of survival and disease progression.

Design  A single-center, retrospective cohort analysis.

Setting  Academic referral center for cutaneous lymphoma.

Patients  Five hundred twenty-five patients with MF and SS evaluated and managed at Stanford University Cutaneous Lymphoma Clinic, Stanford, Calif, from 1958 through 1999.

Main Outcome Measures  We calculated long-term actuarial overall and disease-specific survivals and disease progression by the Kaplan-Meier method, and relative risk (RR) for survival calculated from expected survivals in control populations.

Results  The majority of our patients presented with T1 (30%) or T2 (37%) disease; 18% presented with T3 and 15% with T4 skin involvement. Forty-three percent of deaths were attributable to MF, primarily in patients with T3 or T4 disease. The patients with a more advanced T classification and clinical stage had a worse survival outcome. Except for patients with T1 or stage IA disease, the RR for death is greater in patients with MF than in a control population (RR, 2.2 in stage IB/IIA disease, 3.9 in stage IIB/III disease, and 12.8 in stage IV disease). Despite similar overall survival in patients with stage IB or IIA disease, their disease-specific survivals were significantly different (P = .006). The most significant clinical prognostic factors in the univariate analysis were patient age, TNM and B classifications, overall clinical stage groupings, and the presence or absence of extracutaneous disease. In the multivariate analysis, patient age, T classification, and the presence of extracutaneous disease were the most important independent factors. The risk for disease progression to a more advanced TNM or B classification, worse clinical stage, or death due to MF correlated with the severity of the initial T classification. The risk for development of extracutaneous disease also correlated with T classification; none of these patients had T1 disease when their extracutaneous disease was detected.

Conclusions  Patients with MF and SS have varying risks for disease progression or death. The most important clinical predictive factors for survival include patient age, T classification, and the presence of extracutaneous disease. The significant disease-specific survival differences between different clinical stages validate the usefulness of the present MF clinical staging system of the National Cancer Institute.

From the Departments of Dermatology (Drs Kim, Liu, and Mraz-Gernhard and Ms Varghese) and Radiation Oncology (Dr Hoppe), Stanford University School of Medicine, Stanford, Calif. The authors have no relevant financial interest in this article.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

RELATED ARTICLES

skINsight Starts
James M. Grichnik and Kenneth A. Arndt
Arch Dermatol. 2003;139(7):926.
EXTRACT | FULL TEXT  

Memorials and Mandates for Cutaneous Lymphomas
Peter W. Heald
Arch Dermatol. 2003;139(7):926-928.
EXTRACT | FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

How I treat mycosis fungoides and Sezary syndrome
Prince et al.
Blood 2009;114:4337-4353.
ABSTRACT | FULL TEXT  

Oncogenomic analysis of mycosis fungoides reveals major differences with Sezary syndrome
van Doorn et al.
Blood 2009;113:127-136.
ABSTRACT | FULL TEXT  

Granulomatous Mycosis Fungoides and Granulomatous Slack Skin: A Multicenter Study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization for Research and Treatment of Cancer (EORTC)
Kempf et al.
Arch Dermatol 2008;144:1609-1617.
ABSTRACT | FULL TEXT  

Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation
Arulogun et al.
Blood 2008;112:3082-3087.
ABSTRACT | FULL TEXT  

Lesional gene expression profiling in cutaneous T-cell lymphoma reveals natural clusters associated with disease outcome
Shin et al.
Blood 2007;110:3015-3027.
ABSTRACT | FULL TEXT  

Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)
Olsen et al.
Blood 2007;110:1713-1722.
ABSTRACT | FULL TEXT  

Phase IIB Multicenter Trial of Vorinostat in Patients With Persistent, Progressive, or Treatment Refractory Cutaneous T-Cell Lymphoma
Olsen et al.
JCO 2007;25:3109-3115.
ABSTRACT | FULL TEXT  

TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC)
Kim et al.
Blood 2007;110:479-484.
ABSTRACT | FULL TEXT  

Second Lymphomas and Other Malignant Neoplasms in Patients With Mycosis Fungoides and Sezary Syndrome: Evidence From Population-Based and Clinical Cohorts
Huang et al.
Arch Dermatol 2007;143:45-50.
ABSTRACT | FULL TEXT  

Analysis of T-Cell Receptor Gene Rearrangement for Predicting Clinical Outcome in Patients With Cutaneous T-Cell Lymphoma: A Comparison of Southern Blot and Polymerase Chain Reaction Methods
Juarez et al.
Arch Dermatol 2005;141:1107-1113.
ABSTRACT | FULL TEXT  

Beyond Clonal Detection: Defining the T-Cell Clone
Guitart
Arch Dermatol 2005;141:1159-1160.
FULL TEXT  

WHO-EORTC classification for cutaneous lymphomas
Willemze et al.
Blood 2005;105:3768-3785.
ABSTRACT | FULL TEXT  

Mycosis Fungoides-Type Cutaneous T-Cell Lymphoma and Neutrophilic Dermatosis
Franck et al.
Arch Dermatol 2005;141:353-356.
ABSTRACT | FULL TEXT  

Early TCR-{beta} and TCR-{gamma} PCR detection of T-cell clonality indicates minimal tumor disease in lymph nodes of cutaneous T-cell lymphoma: diagnostic and prognostic implications
Assaf et al.
Blood 2005;105:503-510.
ABSTRACT | FULL TEXT  

Transition of Sezary syndrome into mycosis fungoides after complete clinical and molecular remission under extracorporeal photophoresis
Assaf et al.
J. Clin. Pathol. 2004;57:1325-1328.
ABSTRACT | FULL TEXT  

Clinical and Pathological Features of Posttransplantation Lymphoproliferative Disorders Presenting With Skin Involvement in 4 Patients
Beynet et al.
Arch Dermatol 2004;140:1140-1146.
ABSTRACT | FULL TEXT  

The Pathogenesis of Mycosis Fungoides
Girardi et al.
NEJM 2004;350:1978-1988.
FULL TEXT  

Memorials and Mandates for Cutaneous Lymphomas
Heald
Arch Dermatol 2003;139:926-928.
FULL TEXT