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Observation
February 2014

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

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

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.

An 85-year-old white woman with early dementia presented with 6 months of fatigue, weight loss, and diffuse pruritic violaceous patches and plaques (Figure 1A). Findings from lymph node examination were unremarkable. Biopsies of a patch and plaque on the back revealed a dense bandlike infiltrate of atypical lymphocytes in the dermis with large and irregular forms (Figure 2A). Atypical lymphocytes extended into the epidermis arranged along the dermal-epidermal junction and within the Pautrier microabscesses (Figure 2B). Immunohistochemical staining characterized the lymphocytic population as CD3+ T-lymphocytes with a predominance of CD4+ over CD8+ cells. Expression of CD7 was decreased. Loose aggregates of enlarged dermal lymphocytes, making up approximately 25% of the lymphoid infiltrate, showed CD30 positivity (Figure 2C). Anaplastic lymphoma kinase staining was negative. A complete blood cell count was within normal limits. The lactate dehydrogenase level was normal at 522 U/L. Peripheral flow cytometry revealed immunophenotypically abnormal CD4+ T-cells with reduced CD2 and CD3 expression and loss of CD7 making up 42% of the total lymphocytes. These findings were suggestive of stage IVA (T3B2) SS with large-cell transformation.

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