title: Case of primary cutaneous peripheral T-cell lymphoma, not otherwise specified, with characteristics of follicular helper T cells.
abstract: We report a case of an 88-year-old woman with a decalvant, erythematous, ulcerated tumor extending from the right temporal to occipital region. Histopathological analysis revealed a dense infiltration of medium-to-large-sized atypical cells throughout the entire dermis. The result of immunohistochemical analysis showed that the infiltrating T cells expressed programmed death-1 (PD-1), Bcl-6 and CXCL13. Flow cytometry analysis showed that CD4(+) PD-1(hi) T cells also expressed CD10, inducible T-cell co-stimulator and CXCR5. On the basis of the clinical appearance and the histopathological findings, we diagnosed the patient with primary cutaneous peripheral T-cell lymphoma, not otherwise specified. Recently, the concept of primary cutaneous follicular helper T (TFH)-cell lymphoma was proposed, and in this case, tumor cells clearly expressed TFH-cell markers. Therefore, we considered this case to be a variant of the entity. Although this entity is still provisional, this case supports the new concept.
authors.affiliation: Department of Dermatology, University of Yamanashi, Chuo, Japan.
authors.forename: Machiko
authors.initials: M
authors.lastname: Takaki
authors.forename: Takashi
authors.initials: T
authors.lastname: Inozume
authors.forename: Takamitsu
authors.initials: T
authors.lastname: Matsuzawa
authors.forename: Noriko
authors.initials: N
authors.lastname: Ando
authors.forename: Miyuki
authors.initials: M
authors.lastname: Yamaguchi
authors.forename: Kazutoshi
authors.initials: K
authors.lastname: Harada
authors.forename: Tatsuyoshi
authors.initials: T
authors.lastname: Kawamura
authors.forename: Naotaka
authors.initials: N
authors.lastname: Shibagaki
authors.forename: Shinji
authors.initials: S
authors.lastname: Shimada
mesh_terms: D000369:Aged, 80 and over; D015415:Biomarkers / Q000378:metabolism; D005260:Female; D006258:Head and Neck Neoplasms / Q000150:complications / Q000276:immunology / Q000473:pathology*; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000150:complications / Q000473:pathology*; D012883:Skin Ulcer / Q000209:etiology; D006377:T-Lymphocytes, Helper-Inducer / Q000378:metabolism / Q000473:pathology*
chemical_list: D015415:Biomarkers
keywords: cutaneous T-cell lymphoma; follicular helper T cell; primary cutaneous follicular helper T-cell lymphoma; primary cutaneous peripheral T-cell lymphoma, not otherwise specified; programmed death-1