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原发性皮肤T细胞淋巴瘤中CD4和CD8抗原表达的免疫表型转变:3例临床病理研究

Immunophenotypic shift of CD4 and CD8 antigen expression in primary cutaneous T-cell lymphomas: a clinicopathologic study of three cases.

作者信息

Aung Phyu Phyu, Climent Fina, Muzzafar Tariq, Curry Jonathan L, Patel Keyur P, Servitje Octavio, Prieto Victor G, Duvic Madeleine, Jaffe Elaine S, Torres-Cabala Carlos A

机构信息

National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.

出版信息

J Cutan Pathol. 2014 Jan;41(1):51-7. doi: 10.1111/cup.12252. Epub 2013 Nov 14.

Abstract

Primary cutaneous T-cell lymphomas (CTCL) comprise a heterogeneous group of neoplasms with diverse clinical behavior. Mycosis fungoides (MF) is the most common type of CTCL. Immunophenotypical shift during progression of the disease is a rare event and its significance is unknown. We present three primary CTCL cases that showed an immunophenotypical shift and poor prognosis. Conventional hematoxylin/eosin and immunohistochemical-stained sections were examined in all the cases. Molecular analysis for rearrangement of the T-cell receptor (TCR) gene was performed in two cases. One case was classified as MF, while the other two lacked epidermotropism, and were considered primary cutaneous peripheral T-cell lymphoma (PTCL), NOS. Two cases were CD3+/CD4+ and one case was CD3+/CD8+ at diagnosis. The first two patients suffered many relapses and eventually, new CTCL lesions with a CD3+/CD8+ phenotype were observed. Both cases revealed identical clonal TCR rearrangements on the initial and late lesions, supporting the interpretation of a single clonal proliferation with different phenotypes. The third case progressed with skin recurrences and pulmonary lesions with a predominant CD3+/CD4+/CD8- phenotype. All cases manifested poor prognosis and two patients died of lymphoma. Immunophenotypical shift between CD4 and CD8 in CTCL seems to be a rare phenomenon that may be associated with disease progression.

摘要

原发性皮肤T细胞淋巴瘤(CTCL)是一组具有不同临床行为的异质性肿瘤。蕈样肉芽肿(MF)是CTCL最常见的类型。疾病进展过程中的免疫表型转变是一种罕见事件,其意义尚不清楚。我们报告了3例原发性CTCL病例,这些病例表现出免疫表型转变且预后不良。所有病例均检查了常规苏木精/伊红染色切片和免疫组织化学染色切片。对其中2例进行了T细胞受体(TCR)基因重排的分子分析。1例被分类为MF,另外2例缺乏亲表皮性,被认为是原发性皮肤外周T细胞淋巴瘤(PTCL),NOS。诊断时2例为CD3+/CD4+,1例为CD3+/CD8+。前2例患者多次复发,最终观察到具有CD3+/CD8+表型的新CTCL病变。这两例病例在初始和晚期病变中均显示相同的克隆性TCR重排,支持单一克隆增殖具有不同表型的解释。第3例进展为皮肤复发和肺部病变,主要表型为CD3+/CD4+/CD8-。所有病例预后均较差,2例患者死于淋巴瘤。CTCL中CD4和CD8之间的免疫表型转变似乎是一种罕见现象,可能与疾病进展有关。

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