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Primary CD20+CD10+CD8+ T-Cell Lymphoma of the Skin With Dual IgH and TCRβ Gene Rearrangement

Cynthia M. Magro MD, Kay H. Seilstad MD, Pierluigi Porcu MD, Carl D. Morrison MD
DOI: http://dx.doi.org/10.1309/HPYGFLNXKFBHDQEV 14-22 First published online: 1 July 2006


Most cutaneous T-cell lymphomas are derived from mature postthymic T cells of the CD4 subtype. When other less common profiles are encountered, a diagnostic challenge is posed. Accurate categorization is critical because of the specificity of therapeutic regimens, including biologics. A 65-year-old woman was seen in 2001 because of a thigh mass manifesting an unusual phenotype eventually categorized as a mature postthymic CD8+ T-cell lymphoma with CD10 and weak CD20 expression. Molecular studies revealed T-cell receptor and heavy chain immunoglobulin rearrangement. Her cutaneous disease progressed despite several cycles of chemotherapy and radiation therapy. However, a therapeutic trial with denileukin diftitox resulted in a striking response. The importance of this case lies in the novel phenotype and dual T- and B-cell rearrangements. Rather than representing an aberrant phenotype, this tumor may represent the malignant counterpart of a benign population of weakly CD20+ T cells of the CD8 subset.

Key Words:
  • Primary T-cell lymphoma
  • Skin neoplasms
  • CD20
  • CD10
  • CD8
  • T-cell receptor β
  • Immunoglobulin heavy chain
  • Gene rearrangement