CD4/CD8 Double Negative Mycosis Fungoides With PD-1 (CD279) Expression-A Disease of Follicular Helper T-Cells?

被引:25
作者
Kempf, Werner [1 ,2 ]
Kazakov, Dmitry V. [3 ]
Cipolat, Claudio
Kutzner, Heinz [4 ]
Roncador, Giovanna [5 ]
Tomasini, Dario [6 ]
机构
[1] Kempf & Pfaltz, Histol Diagnost, CH-8042 Zurich, Switzerland
[2] Univ Zurich Hosp, Dept Dermatol, CH-8091 Zurich, Switzerland
[3] Charles Univ Prague, Fac Med Pilsen, Dept Pathol, Pilseu, Czech Republic
[4] Dermatopathol Friedrichshafen Bodensee, Friedrichshafen, Germany
[5] Spanish Natl Canc Res Ctr, CNIO, Comparat Pathol Unit, Madrid, Spain
[6] Hosp Busto Arsizio, Dept Dermatol, Busto Arsizio, Italy
关键词
mycosis fungoides; cutaneous T-cell lymphoma; CD8; PD-1; PROGRAMMED DEATH-1; PAGETOID RETICULOSIS; LYMPHOMA; FEATURES; PROFILE; GENES;
D O I
10.1097/DAD.0b013e31825b26d1
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
CD4/CD8 double negative mycosis fungoides (MF) is a rare phenotypic variant of this epidermotropic cutaneous T-cell lymphoma. Clinically, this MF form manifests with unusual appearances such as annular lesions confined to one body region as in our patient in whom the lesions were found on the left lower leg. The cellular origin of CD4/CD8 double negative MF is unknown. In our case, the intraepidermal CD4/CD8 double negative clonal T-lymphocytes (CD2+, CD4-, CD8-, CD30-, beta-F1+) expressed programmed death-1 but were negative for CXCL-13 and cytotoxic molecules (TIA-1, granzyme B, perforin). Our observation may give an insight into the histogenesis of this unique MF variant and may also be of therapeutic significance because programmed death-1 may serve as a target for therapeutic intervention.
引用
收藏
页码:757 / 761
页数:5
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