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Mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the literature and study of the clinical course.

Authors :
Quaglino P
Zaccagna A
Verrone A
Dardano F
Bernengo MG
Source :
Dermatology (Basel, Switzerland) [Dermatology] 1999; Vol. 199 (1), pp. 8-14.
Publication Year :
1999

Abstract

Background: Mycosis fungoides (MF) is rare in young patients. Its clinical behavior is still uncertain, as some reports have suggested that it has a more aggressive course than does the adult-onset type.<br />Aim: To ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas.<br />Materials and Methods: Clinical, immunohistopathological and follow-up data of early-onset (<20 years of age) MF cases reported in the literature (n = 42) plus 7 described herein were compared with those of a cohort of adult-onset MF patients (n = 252) diagnosed at our institution since 1975.<br />Results: The majority of the 49 early-onset MF patients had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The predominant phenotype was CD3+ CD4+CD7-CD8-. Seven patients had a stage progression, 6 with extracutaneous involvement. Five- and 10-year survival rates were 93 and 74%, respectively.<br />Conclusions: No statistically significant differences were found in the disease course between early- and adult-onset MF.

Details

Language :
English
ISSN :
1018-8665
Volume :
199
Issue :
1
Database :
MEDLINE
Journal :
Dermatology (Basel, Switzerland)
Publication Type :
Academic Journal
Accession number :
10449950
Full Text :
https://doi.org/10.1159/000018196