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Primary cutaneous lymphoma: Two-decade comparison in a population of 263 cases from a Swiss tertiary referral centre


Jenni, D; Karpova, M B; Seifert, Burkhardt; Golling, P; Cozzio, A; Kempf, W; French, L E; Dummer, R (2011). Primary cutaneous lymphoma: Two-decade comparison in a population of 263 cases from a Swiss tertiary referral centre. British Journal of Dermatology, 164(5):1071-1077.

Abstract

Background:  Epidemiologic data on primary cutaneous lymphomas (PCL) are rare and up to now have not been investigated in Switzerland. Objective:  To analyze variations in demographics, pattern of subtypes and staging during the two ten-year intervals: 1990-1999 and 2000-2009. Methods:  Descriptive study of 263 PCL patients based on a retrospective review and reassessment according to the WHO-EORTC classification. Results:  Change was observed in the pattern of cutaneous T-cell lymphoma subtypes: Sézary Syndrome frequency decreased (17-7%); CD30+ lymphoproliferative disorders frequency increased (7-18%; common p=0.04). Staging of PCL showed a higher number of early stage Mycosis fungoides cases (p=0.01). In relation to the international data, Zurich showed a higher number of Sézary Syndrome (11% vs. 3%) and Marginal Cell Lymphoma (14% vs. 5-7%) patients. Additionally, comparison of the survival data showed prolonged median overall survival of Zurich Sézary Syndrome patients in the second ten-year interval (6.5 vs. 2-4 years). Conclusion:  The increasing frequency of marginal cell lymphoma and CD30+ lymphoproliferative disorders might depend on an increased awareness of these diseases in the medical community driven by progress in the classification and staging of these disease entities.

Background:  Epidemiologic data on primary cutaneous lymphomas (PCL) are rare and up to now have not been investigated in Switzerland. Objective:  To analyze variations in demographics, pattern of subtypes and staging during the two ten-year intervals: 1990-1999 and 2000-2009. Methods:  Descriptive study of 263 PCL patients based on a retrospective review and reassessment according to the WHO-EORTC classification. Results:  Change was observed in the pattern of cutaneous T-cell lymphoma subtypes: Sézary Syndrome frequency decreased (17-7%); CD30+ lymphoproliferative disorders frequency increased (7-18%; common p=0.04). Staging of PCL showed a higher number of early stage Mycosis fungoides cases (p=0.01). In relation to the international data, Zurich showed a higher number of Sézary Syndrome (11% vs. 3%) and Marginal Cell Lymphoma (14% vs. 5-7%) patients. Additionally, comparison of the survival data showed prolonged median overall survival of Zurich Sézary Syndrome patients in the second ten-year interval (6.5 vs. 2-4 years). Conclusion:  The increasing frequency of marginal cell lymphoma and CD30+ lymphoproliferative disorders might depend on an increased awareness of these diseases in the medical community driven by progress in the classification and staging of these disease entities.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Epidemiology, Biostatistics and Prevention Institute (EBPI)
04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2011
Deposited On:09 Jan 2011 17:30
Last Modified:05 Apr 2016 14:32
Publisher:Wiley-Blackwell
ISSN:0007-0963
Publisher DOI:10.1111/j.1365-2133.2010.10143.x
PubMed ID:21083546
Permanent URL: http://doi.org/10.5167/uzh-41556

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