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皮肤原发性CD20+CD10+CD8+ T细胞淋巴瘤伴免疫球蛋白重链(IgH)和T细胞受体β(TCRβ)基因重排

Primary CD20+CD10+CD8+ T-cell lymphoma of the skin with IgH and TCR beta gene rearrangement.

作者信息

Magro Cynthia M, Seilstad Kay H, Porcu Pierluigi, Morrison Carl D

机构信息

Department of Pathology, The Ohio State University, Columbus, USA.

出版信息

Am J Clin Pathol. 2006 Jul;126(1):14-22. doi: 10.1309/HPYG-FLNX-KFBH-DQEV.

DOI:10.1309/HPYG-FLNX-KFBH-DQEV
PMID:16753590
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.

摘要

大多数皮肤T细胞淋巴瘤源自CD4亚型的成熟胸腺后T细胞。当遇到其他不太常见的情况时,就会带来诊断挑战。由于治疗方案(包括生物制剂)的特异性,准确分类至关重要。2001年,一名65岁女性因大腿肿物就诊,该肿物表现出一种不寻常的表型,最终被归类为具有CD10和弱CD20表达的成熟胸腺后CD8 + T细胞淋巴瘤。分子研究显示T细胞受体和重链免疫球蛋白重排。尽管进行了几个周期的化疗和放疗,她的皮肤疾病仍进展。然而,用地尼白介素妥西罗汀进行的治疗试验产生了显著反应。该病例的重要性在于其新的表型以及T细胞和B细胞的双重重排。这种肿瘤可能代表CD8亚群中弱CD20 + T细胞良性群体的恶性对应物,而不是代表一种异常表型。

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