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

Kempf, Werner MD*,†; Kazakov, Dmitry V. MD, PhD; Cipolat, Claudio MD§; Kutzner, Heinz MD; Roncador, Giovanna MD, PhD; Tomasini, Dario MD**

The American Journal of Dermatopathology: October 2012 - Volume 34 - Issue 7 - p 757–761
doi: 10.1097/DAD.0b013e31825b26d1
Extraordinary Case Report

Abstract: 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.

*Kempf und Pfaltz, Histologische Diagnostik

Department of Dermatology, University Hospital, Zürich, Switzerland

Department of Pathology, Faculty of Medicine in Pilsen, Charles University in Prague, Czech Republic, Pilseu

§Dermatology Practice, Dübendorf, Switzerland

Dermatopathologie Friedrichshafen Bodensee, Friedrichshafen, Germany

Comparative Pathology Unit, Biotechnology program, Spanish National Cancer Research Centre (CNIO) Centro Nacional de Investigaciones Oncologicas, Madrid, Spain

**Department of Dermatology, Hospital of Busto Arsizio, Busto Arsizio, Italy.

Reprints: Werner Kempf, MD, Kempf und Pfaltz, Histologische Diagnostik, Seminarstrasse 1, CH-8042, Zurich, Switzerland (e-mail:

The authors declare no conflicts of interest.

© 2012 Lippincott Williams & Wilkins, Inc.