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

• Published in: Acta dermato-venereologica Vol. 97; no. 9; pp. 1081 - 1086
• Main Authors: Mantaka, Panagiota, Malecka, Agnieszka, Trøen, Gunhild, Helsing, Per, Gjersvik, Petter, Beiske, Klaus, Delabie, Jan
• Format: Journal Article
• Language: English
• Published: Sweden Taylor & Francis 02-10-2017; Medical Journals Sweden
• Subjects: Antigens, CD1 - analysis; Case-Control Studies; CD1; Complementarity Determining Regions - analysis; complementarity-determiningregion3; cutaneousT-celllymphoma; Dendritic Cells - chemistry; folliculotropicmycosisfungoides; gene; Genes, T-Cell Receptor beta; Glycoproteins - analysis; Hair Follicle - chemistry; Hair Follicle - pathology; Humans; Immunohistochemistry; Lymphoma, T-Cell, Cutaneous - chemistry; Lymphoma, T-Cell, Cutaneous - genetics; Lymphoma, T-Cell, Cutaneous - pathology; Mycosis Fungoides - chemistry; Mycosis Fungoides - genetics; Mycosis Fungoides - pathology; mycosisfungoides; Skin Neoplasms - chemistry; Skin Neoplasms - genetics; Skin Neoplasms - pathology; T-cellreceptorβ
• ISSN: 0001-5555; 1651-2057
• DOI: 10.2340/00015555-2722

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.