Skip Navigation LinksHome > October 2012 - Volume 34 - Issue 7 > CD4/CD8 Double Negative Mycosis Fungoides With PD-1 (CD279)...
American Journal of Dermatopathology:
doi: 10.1097/DAD.0b013e31825b26d1
Extraordinary Case Report

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**

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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.

© 2012 Lippincott Williams & Wilkins, Inc.


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