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A Case of Refractory Sézary Syndrome With Large-Cell Transformation Responsive to Brentuximab Vedotin

Kristen Corey, BS1; Deborah Cook, MD2; Jessica Bekker, MD2; Emiliano Mugnaini, MD, PhD3; Julie H. Lin, MD4
[+] Author Affiliations
1University of Massachusetts Medical School, Worcester
2Department of Pathology, University of Vermont College of Medicine, Burlington
3Division of Hematology/Oncology, University of Vermont College of Medicine, Burlington
4Division of Dermatology, University of Vermont College of Medicine, Burlington
JAMA Dermatol. 2014;150(2):210-212. doi:10.1001/jamadermatol.2013.5741.
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Brentuximab vedotin is a CD30-directed antibody/drug conjugate recently approved for the treatment of relapsed Hodgkin lymphoma (HL) and systemic anaplastic large-cell lymphoma (ALCL). Given that CD30 is variably expressed in mycosis fungoides (MF) and Sézary syndrome (SS), brentuximab vedotin is a promising treatment option for these cutaneous neoplasms. Initial studies have confirmed its clinical activity in refractory cases.

Article InformationCorresponding Author: Julie H. Lin, MD, Division of Dermatology, University of Vermont College of Medicine, 111 Colchester Ave, Burlington, VT 05401 (Julie.Lin@vtmednet.org).

Published Online: December 18, 2013. doi:10.1001/jamadermatol.2013.5741.

Conflict of Interest Disclosures: None reported.

Additional Contributions: We would like to thank Laura McGevna, MD, of the Division of Dermatology, and Paul Larson, NP, and Barbara Grant, MD, of the Division of Hematology/Oncology at University of Vermont College of Medicine for their help and expertise in caring for our patient. We would also like to thank the patient and her family for allowing us to publish this information.

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Figure 1.
Clinical Images of the Patient

A, Photograph of the patient at initial presentation. B, Rapid progression seen 6 months later despite multiple therapies. C, Rapid clearance of tumors and improvement of pruritus 10 months later after 5 treatment cycles of brentuximab vedotin.

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Figure 2.
Pathologic and Immunohistochemical Specimens

A, Skin biopsy specimen taken at initial presentation shows dense bandlike infiltrate of atypical lymphocytes (hematoxylin-eosin, original magnification ×10). B, Higher magnification (original magnification ×40) shows large cells with atypia (hematoxylin-eosin). Immunohistochemical staining shows CD30 positivity (original magnification ×40).

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