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Differential Expression of Programmed Death-1 (PD-1) in Sézary Syndrome and Mycosis Fungoides

Fatma Çetinözman, MD; Patty M. Jansen, MD, PhD; Maarten H. Vermeer, MD, PhD; Rein Willemze, MD, PhD
Arch Dermatol. 2012;148(12):1379-1385. doi:10.1001/archdermatol.2012.2089.
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Objective  To determine if there are differences in the expression of programmed death-1 (PD-1) between SS and MF, and in particular erythrodermic MF (E-MF). PD-1 is a marker of follicular helper T (TFH) cells and is expressed by the neoplastic T cells of some types of malignant T-cell lymphoma, including mycosis fungoides (MF) and Sézary syndrome (SS). Reported results of PD-1 staining in MF and SS are, however, conflicting.

Design  Formalin-fixed, paraffin-embedded skin biopsy specimens were stained for PD-1. In addition, PD-1+ cases were stained with antibodies against BCL6, CXCL13, and CD10 to find possible relationship with TFH cells.

Setting  Tertiary referral center for cutaneous lymphomas.

Patients  Twenty-seven patients with SS and 60 patients with MF, including 8 patients with E-MF.

Results  In patients with SS, expression of PD-1 by more than 50% of the neoplastic T cells was observed in 24 of 27 cases (89%). In contrast, PD-1 expression by more than 50% of neoplastic T cells was found in only 8 of 60 patients with MF (13%), including only 1 of 8 patients with E-MF (12%). In PD-1+ cases, serial skin sections showed that CXCL13 and BCL6 generally stained 25% to 50% of the PD-1+ cells, while expression of CD10 was uncommon.

Conclusion  The results of the present study show differential expression of PD-1 between SS and MF/E-MF, which provides further support for the view that SS and MF are distinct entities.

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Figures

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Grahic Jump Location

Figure 1. Histopathologic features of a representative patient with Sézary syndrome. The hematoxylin-eosin staining of the lesion showed a superficial bandlike dermal infiltrate and epidermal Pautrier microabscesses (A) with CD4+ (B) and CD3+ (C) neoplastic T cells. These neoplastic T cells showed strong PD-1 positivity (D), and serial skin sections showed that BCL6 (E) and CXCL13 (F) was expressed by 25% to 50% of the PD-1+ cells. A-D, Original magnification ×100. E and F, Original magnification ×400. Insets (in A and D), original magnification ×2.

Place holder to copy figure label and caption
Grahic Jump Location

Figure 2. Histopathologic features of a representative patient who was negative for programmed death-1 (PD-1) with mycosis fungoides. The hematoxylin-eosin staining (original magnification ×100) of the lesion showed an epidermal and superficial dermal infiltrate (A) with CD4+ (B) and CD3+ (C) neoplastic T cells. PD-1 expression was seen in less than 10% of the tumor cells (D).

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