Folliculotropic Mycosis Fungoides with Skewed T-cell Receptor CDR3 Motif: Suggestive of Lipid-antigen Selection?

• Main Authors: Panagiota Mantaka, Agnieszka Malecka, Gunhild Trøen, Per Helsing, Petter Gjersvik, Klaus Beiske, Jan Delabie
• Format: Article
• Language: English
• Published: Society for Publication of Acta Dermato-Venereologica 2017-06-01
• Series: Acta Dermato-Venereologica
• Subjects: folliculotropicmycosisfungoides; mycosisfungoides; cutaneousT-celllymphoma; T-cellreceptorβ; gene; complementarity-determiningregion3; CD1
• Online Access: https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2722
• ISSN: 0001-5555; 1651-2057

Folliculotropic mycosis fungoides (FMF), a variant of mycosis fungoides (MF) with distinct clinical features, is characterized by infiltration of malignant T cells in hair follicles. This raises the hypothesis that antigens in the hair follicle may contribute to the pathogenesis of FMF. T-cell receptor β gene (TRB) sequences as well as dendritic cell subsets in patients with FMF (n = 21) and control patients with MF (n = 20) were studied to explore this hypothesis. A recurrent usage of the TRB junctional genes TRBJ2-1 and TRBJ2-7 was found in patients with FMF compared with those with MF. These genes contribute to an amino acid motif in the complementarity-determining region 3 (CDR3) of the T-cell receptor. This motif was previously found in T cells stimulated by lipids bound to CD1 on antigen-presenting cells. Additional immunohistochemical analysis revealed abundant CD1c- and CD1a- expressing dendritic cells in FMF. The combined findings support a role for lipid-antigen stimulation in FMF.