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Prognostic factors and prediction of prognosis by the CTCL Severity Index in mycosis fungoides and Sézary syndrome.
Br J Dermatol 2005; 153(1):118-24BJ

Abstract

BACKGROUND

Cutaneous T-cell lymphoma (CTCL) is a slowly progressive malignancy for which there is no cure. Therefore, accurate prediction of prognosis is important for the conduct of clinical trials and for counselling of individuals.

OBJECTIVES

To improve prediction of survival in patients with CTCL.

METHODS

Prognostic factors including tumour-node-metastasis (TNM) criteria and the CTCL Severity Index (CTCL-SI) were analysed using a Weibull model for multivariate analysis in a sample of 62 patients with classical CTCL (mycosis fungoides and Sézary syndrome). The Brier score was used to quantify the quality of individual prediction.

RESULTS

Estimated 5-year survival rate (SR5) differed according to TNM stage: stage IA, 100% (95% confidence interval 70-100%); IB-III, 86% (73-100%); IVA, 54% (32-91%); IVB, 0% (0-52%). In a multivariate analysis, two independent prognostic factors were identified: lymph node (P = 0.036) and blood involvement (P = 0.015). A probability of survival model showed correlation of CTCL-SI with survival in patients with CTCL-SI > 20 according to the following formula: SR5 = 124-2 x (CTCL-SI)%. Calibration of SR5 against CTCL-SI-independent CTCL subsets revealed underestimation of Sézary syndrome. When CTCL-SI parameters were adjusted accordingly, the probability of survival model did not change significantly, while SR5 values became adequate. In addition, CTCL-SI was shown to be superior to TNM by 30% regarding individual predictive power.

CONCLUSIONS

Probability of survival in CTCL can be accurately predicted by a CTCL-SI-based survival rate formula. Careful monitoring of lymph node and blood compartments and quantification by CTCL-SI are reliable tools for follow-up of patients with CTCL and allow progression-adjusted prediction of prognosis.

Authors+Show Affiliations

Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Theodor-Kutzer Ufer 1-3, 68135 Mannheim, Germany. claus-detlev.klemke@haut.ma.uni-heidelberg.deNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Multicenter Study

Language

eng

PubMed ID

16029336

Citation

Klemke, C-D, et al. "Prognostic Factors and Prediction of Prognosis By the CTCL Severity Index in Mycosis Fungoides and Sézary Syndrome." The British Journal of Dermatology, vol. 153, no. 1, 2005, pp. 118-24.
Klemke CD, Mansmann U, Poenitz N, et al. Prognostic factors and prediction of prognosis by the CTCL Severity Index in mycosis fungoides and Sézary syndrome. Br J Dermatol. 2005;153(1):118-24.
Klemke, C. D., Mansmann, U., Poenitz, N., Dippel, E., & Goerdt, S. (2005). Prognostic factors and prediction of prognosis by the CTCL Severity Index in mycosis fungoides and Sézary syndrome. The British Journal of Dermatology, 153(1), pp. 118-24.
Klemke CD, et al. Prognostic Factors and Prediction of Prognosis By the CTCL Severity Index in Mycosis Fungoides and Sézary Syndrome. Br J Dermatol. 2005;153(1):118-24. PubMed PMID: 16029336.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Prognostic factors and prediction of prognosis by the CTCL Severity Index in mycosis fungoides and Sézary syndrome. AU - Klemke,C-D, AU - Mansmann,U, AU - Poenitz,N, AU - Dippel,E, AU - Goerdt,S, PY - 2005/7/21/pubmed PY - 2005/10/26/medline PY - 2005/7/21/entrez SP - 118 EP - 24 JF - The British journal of dermatology JO - Br. J. Dermatol. VL - 153 IS - 1 N2 - BACKGROUND: Cutaneous T-cell lymphoma (CTCL) is a slowly progressive malignancy for which there is no cure. Therefore, accurate prediction of prognosis is important for the conduct of clinical trials and for counselling of individuals. OBJECTIVES: To improve prediction of survival in patients with CTCL. METHODS: Prognostic factors including tumour-node-metastasis (TNM) criteria and the CTCL Severity Index (CTCL-SI) were analysed using a Weibull model for multivariate analysis in a sample of 62 patients with classical CTCL (mycosis fungoides and Sézary syndrome). The Brier score was used to quantify the quality of individual prediction. RESULTS: Estimated 5-year survival rate (SR5) differed according to TNM stage: stage IA, 100% (95% confidence interval 70-100%); IB-III, 86% (73-100%); IVA, 54% (32-91%); IVB, 0% (0-52%). In a multivariate analysis, two independent prognostic factors were identified: lymph node (P = 0.036) and blood involvement (P = 0.015). A probability of survival model showed correlation of CTCL-SI with survival in patients with CTCL-SI > 20 according to the following formula: SR5 = 124-2 x (CTCL-SI)%. Calibration of SR5 against CTCL-SI-independent CTCL subsets revealed underestimation of Sézary syndrome. When CTCL-SI parameters were adjusted accordingly, the probability of survival model did not change significantly, while SR5 values became adequate. In addition, CTCL-SI was shown to be superior to TNM by 30% regarding individual predictive power. CONCLUSIONS: Probability of survival in CTCL can be accurately predicted by a CTCL-SI-based survival rate formula. Careful monitoring of lymph node and blood compartments and quantification by CTCL-SI are reliable tools for follow-up of patients with CTCL and allow progression-adjusted prediction of prognosis. SN - 0007-0963 UR - https://www.unboundmedicine.com/medline/citation/16029336/Prognostic_factors_and_prediction_of_prognosis_by_the_CTCL_Severity_Index_in_mycosis_fungoides_and_Sézary_syndrome_ L2 - https://doi.org/10.1111/j.1365-2133.2005.06676.x DB - PRIME DP - Unbound Medicine ER -