Pediatric Mycosis Fungoides: Retrospective Analysis of a Series With CD8 + Profile and Female Predominance
- PMID: 34699461
- DOI: 10.1097/MPH.0000000000002354
Pediatric Mycosis Fungoides: Retrospective Analysis of a Series With CD8 + Profile and Female Predominance
Abstract
Background: Mycosis fungoides (MF) in children is a rare disease and there are limited data regarding the behavior of the disease in this age group. We aimed to collect additional data to better understand the clinicopathologic features of MF in children.
Materials and methods: This study was a retrospective analysis of pediatric MF patients (diagnosed at age 0 to 18 y).
Results: Thirteen pediatric patients with MF were identified. Female predominance was observed with a ratio of 1.6:1. Median values for age of onset of skin lesions and age at the time of histologic diagnosis were 5 and 12 years, respectively. All patients had early stage (stage IA to IIA) of MF at the time of diagnosis. Hypopigmented MF comprised 77% of all study patients, followed by classic MF (15%) and pagetoid reticulosis (8%). The lower extremity (especially proximal leg) followed by trunk and upper extremity were most commonly affected sites. Seven of 9 patients who had available immunohistochemistry data showed CD8 + predominance. Five of 8 patients whose follow-up data was available, achieved complete response with narrowband ultraviolet B treatment, while 2 and 1 had near complete response and partial response, respectively.
Conclusions: Our study demonstrated female sex and CD8 + profile predominance. Hypopigmented MF constituted the majority of cases. We observed good responses with narrowband ultraviolet B treatment.
Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
Conflict of interest statement
S.P. was a recipient of TUBITAK scholarship (2015–2016), Ankara, Turkey. The other author declares no conflict of interest.
References
-
- Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–3785.
-
- Yazganoglu KD, Topkarci Z, Buyukbabani N, et al. Childhood mycosis fungoides: a report of 20 cases from Turkey. J Eur Acad Dermatol Venereol. 2013;27:295–300.
-
- Heng YK, Koh MJ, Giam YC, et al. Pediatric mycosis fungoides in Singapore: a series of 46 children. Pediatr Dermatol. 2014;31:477–482.
-
- Boulos S, Vaid R, Aladily TN, et al. Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: a case series of 34 patients. J Am Acad Dermatol. 2014;71:1117–1126.
-
- Hodak E, Amitay-Laish I, Feinmesser M, et al. Juvenile mycosis fungoides: cutaneous T-cell lymphoma with frequent follicular involvement. J Am Acad Dermatol. 2014;70:993–1001.
MeSH terms
LinkOut - more resources
Full Text Sources
Medical
Research Materials