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

Abstract

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.

Primary Cutaneous Peripheral T-Cell Lymphoma With Aberrant Coexpression of CD20: Case Report and Review of the Literature

“…The expression of TIA-1 and CD7 was not reported in this case. 10 Recently, Sen et al 11 reported a case of mycosis fungoides in the lower extremity that expressed CD20. Similar to the current case, a clonal T-cell receptor g-chain gene rearrangement was present, but no rearrangements of the immunoglobulin heavy or light chain genes were found, and there was lack of CD7 expression.…”

Rare lymphoid neoplasms coexpressing B- and T-cell antigens. The role of PAX-5 gene methylation in their pathogenesis

“…This case expressed the T-cell-associated antigens CD3 and CD8 and had a clonal T-cell receptor (TCR) γ chain rearrangement without evidence of clonal rearrangements of immunoglobulin genes. More recently, a case of primary T-cell lymphoma of the skin with coexpression of CD20 and dual immunoglobulin and TCR gene rearrangement has been reported [15].…”

CD20-positive primary gastric T-cell lymphoma poorly responding to initial treatment with rituximab plus CHOP, and a literature review

“…Among 37 cases, 12 cases underwent chemotherapy as a first-line therapy, according to the limited description of literatures, and interestingly, rituximab was administered in 10 out of the 12 cases. Thus, rituximab has been administered in 11 patients with CD20-positive PTCL-NOS, including our case (Table 1) [4,18,21,[25][26][27][28][29]. Among 11 cases, 8 (72.7 %) occurred in males, and the number of patients at 65 or more than 65 years of age was 8.…”

“…However, in our case, the sample size through endoscopy was inadequate to flow cytometric analysis. In our knowledge, only one case of CD20-positive PTLC with the dual rearrangement has been reported [18].…”

“…In monkeys, its expression on lymph node-derived T lymphocytes was increased when exposed to mitogen and interleukin-2, suggesting that its expression, regardless of aberrant expression or malignant transformation of the normal counterpart, may be associated with a state of activation [18]. Moreover, Matnani et al [21] suggest that the aberrant expression of B cell markers in PTCL represents a higher degree of genetic dysregulation.…”