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Cutaneous Gamma-Delta T-Cell Lymphoma Successfully Treated With Brentuximab Vedotin ONLINE FIRST

Belen Rubio-Gonzalez, MD1; Jasmine Zain, MD2; Lino Garcia, MS3; Steven T. Rosen, MD2; Christiane Querfeld, MD, PhD1,2,4
[+] Author Affiliations
1Department of Pathology, City of Hope, Duarte, California
2Department of Hematology/Hematopoietic Cell Transplantation, City of Hope, Duarte, California
3Division of Cytogenetics, City of Hope, Duarte, California
4Department of Surgery, Division of Dermatology, City of Hope, Duarte, California
JAMA Dermatol. Published online September 21, 2016. doi:10.1001/jamadermatol.2016.3117
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This case report describes a case of cutaneous gamma-delta T-cell lymphoma successfully treated with brentuximab vedotin.

Primary cutaneous gamma-delta T-cell lymphoma (GD-TCL) is a rare skin lymphoma with numerous clinical presentations. Cytotoxic features on histologic analysis and the T-cell receptor (TCR)-gamma expression on immunohistochemical analysis are clues that should raise suspicion for the diagnosis. Expression of CD30 can also be seen in some cases and should be evaluated because it allows for patients to be treated with brentuximab, as in the present case.

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Figure 1.
Clinical Findings of Epidermotropic Cutaneous Gamma-Delta T-Cell Lymphoma and Clinical Response to Brentuximab

A, Multiple necrotic papules and small scaly plaques on the anterior trunk. B, Partial clearing of skin lesions after 4 cycles of brentuximab vedotin treatment.

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Figure 2.
Histopathologic and Fluorescence in Situ Hybridization (FISH) Images

A, Epidermal infiltration of atypical large pleomorphic lymphocytes with associated dyskeratotic and apoptotic keratinocytes and partial ulceration; the underlying superficial dermis shows a bandlike lymphohistiocytic infiltrate. B, Detail of the large cells with clumped chromatin. C, Most of the large cells show T-cell receptor (TCR)-gamma expression by immunohistochemical analysis. D, The results of FISH analysis using X and Y probes (green and red spots, respectively) revealed that the atypical lymphoid cells in epidermis (above the curved line) showed a male (XY) host pattern, while dermal cells (below the curved line) showed a predominant female (XX) donor pattern with scattered male (XY) positive cells.

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