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Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical prognostic factors and risk for disease progression.
Arch Dermatol 2003; 139(7):857-66AD

Abstract

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.

Authors+Show Affiliations

Department of Dermatology, Stanford University School of Medicine, Stanford, CA 94305, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

12873880

Citation

Kim, Youn H., et al. "Long-term Outcome of 525 Patients With Mycosis Fungoides and Sezary Syndrome: Clinical Prognostic Factors and Risk for Disease Progression." Archives of Dermatology, vol. 139, no. 7, 2003, pp. 857-66.
Kim YH, Liu HL, Mraz-Gernhard S, et al. Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical prognostic factors and risk for disease progression. Arch Dermatol. 2003;139(7):857-66.
Kim, Y. H., Liu, H. L., Mraz-Gernhard, S., Varghese, A., & Hoppe, R. T. (2003). Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical prognostic factors and risk for disease progression. Archives of Dermatology, 139(7), pp. 857-66.
Kim YH, et al. Long-term Outcome of 525 Patients With Mycosis Fungoides and Sezary Syndrome: Clinical Prognostic Factors and Risk for Disease Progression. Arch Dermatol. 2003;139(7):857-66. PubMed PMID: 12873880.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical prognostic factors and risk for disease progression. AU - Kim,Youn H, AU - Liu,Howard L, AU - Mraz-Gernhard,Serena, AU - Varghese,Anna, AU - Hoppe,Richard T, PY - 2003/7/23/pubmed PY - 2003/10/8/medline PY - 2003/7/23/entrez SP - 857 EP - 66 JF - Archives of dermatology JO - Arch Dermatol VL - 139 IS - 7 N2 - 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. SN - 0003-987X UR - https://www.unboundmedicine.com/medline/citation/12873880/Long_term_outcome_of_525_patients_with_mycosis_fungoides_and_Sezary_syndrome:_clinical_prognostic_factors_and_risk_for_disease_progression_ L2 - https://jamanetwork.com/journals/jamadermatology/fullarticle/vol/139/pg/857 DB - PRIME DP - Unbound Medicine ER -