Primary Cutaneous T-cell Lymphoma With Coexpression of T-Cell Receptors αβ and γδ

被引:2
|
作者
Parekh, Vishwas [1 ]
Shim, Eun-Hee [2 ]
Knapp, Charles F., III [3 ]
Hughey, Lauren [3 ]
Elmets, Craig A. [3 ]
McKay, Kristopher [1 ,3 ]
机构
[1] Univ Alabama Birmingham, Dept Pathol, Birmingham, AL 35233 USA
[2] Univ Alabama Birmingham, Dept Urol, Birmingham, AL 35233 USA
[3] Univ Alabama Birmingham, Dept Dermatol, Birmingham, AL 35233 USA
关键词
CD4; CD8; T-cell receptor; double-positive; cutaneous lymphoma; MYCOSIS-FUNGOIDES; PERIPHERAL-BLOOD; LYMPHOCYTES; LEUKEMIA; LEUKEMIA/LYMPHOMA;
D O I
10.1097/DAD.0000000000000355
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
T lymphocytes belong to 2 distinct sublineages that express either or T-cell receptor (TCR) complex. Although malignancy is a great instigator of lineage infidelity, as exemplified by aberrant expression of numerous lineage markers in lymphoma cells, malignant T cells rarely coexpress and TCR complexes. Similarly, only rare cases of CD4/CD8 double-positive primary cutaneous T-cell lymphoma have been reported. In this report, we describe a remarkable case of primary cutaneous T-cell lymphoma coexpressing and TCR complexes, strong diffuse CD8, and a very restricted coexpression of CD4 and CD8. A 66-year-old man was referred to our center for treatment of a persistent eczematoid eruption of 6 years of duration. An initial biopsy demonstrated not only marked spongiosis, but also an epidermotropic population of CD4(+) small mature T cells with partial expression of CD8. The process remained indolent for another year, followed by an abrupt progression with development of plaques and tumors. Repeat biopsies of these lesions demonstrated a superimposed population of large anaplastic T cells extensively involving the dermis and epidermis. The large cells showed a strong uniform expression of CD3, CD8, CD45RA, CD5, granzyme, TIA1, perforin, TCR-, and TCR- and a weaker but unambiguous expression of CD4, CD25, CD2, and CD56. TCR gene rearrangement studies showed clonal rearrangements for TCR- and TCR- with identical peaks to those seen in the biopsy from a year earlier. The patient developed lymphadenopathy, with a biopsy showing nodal involvement by a morphologically and phenotypically identical neoplastic T-cell population. The disease showed partial response to systemic chemotherapy with development of new plaques, but these new lesions have regressed with radiation therapy.
引用
收藏
页码:66 / 72
页数:7
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