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获得性免疫缺陷综合征中T细胞表型的原发性肝间变性大细胞淋巴瘤:一例尸检病例报告及文献复习

Primary hepatic anaplastic large-cell lymphoma of T-cell phenotype in acquired immunodeficiency syndrome: a report of an autopsy case and review of the literature.

作者信息

Baschinsky D Y, Weidner N, Baker P B, Frankel W L

机构信息

Department of Pathology, Ohio State University Medical Center and Arthur G. James Cancer Hospital and Research Institute, Columbus, USA.

出版信息

Am J Gastroenterol. 2001 Jan;96(1):227-32. doi: 10.1111/j.1572-0241.2001.03481.x.

Abstract

Anaplastic large-cell lymphomas (ALCL) were first described by Stein et al. in 1985 as large-cell neoplasms with a pleomorphic appearance, subtotal effacement of the lymph node structure, and expression of the lymphoid activation antigen CD-30 (Ki-l). Since their first description, these tumors have been documented in a variety of extranodal sites. We report a primary hepatic anaplastic large-cell lymphoma in a patient with advanced AIDS, who presented with hepatic failure and multiple nodules in the liver. A complete autopsy showed discrete tumor nodules throughout the entire liver without gross or microscopic involvement of lymph nodes or any other organs by the neoplastic process. The tumor cells showed typical histological and immunohistochemical features of ALCL and were strongly immunoreactive with the T-cell markers CD-3 and UCHL-1. Only one previous case of primary hepatic ALCL has been reported in the literature, and this tumor occurred in an immunocompetent patient and was not immunoreactive for B- or T-cell markers. To our knowledge, this study represents the first reported case of primary hepatic anaplastic large-cell lymphoma of T-cell phenotype. Additionally, this is the first case of primary hepatic ALCL reported in an AIDS patient.

摘要

间变性大细胞淋巴瘤(ALCL)于1985年由斯坦因等人首次描述,是一种具有多形性外观、淋巴结结构部分消失以及淋巴激活抗原CD-30(Ki-l)表达的大细胞肿瘤。自首次描述以来,这些肿瘤已在多种结外部位被记录。我们报告了一例晚期艾滋病患者的原发性肝脏间变性大细胞淋巴瘤,该患者表现为肝衰竭和肝脏多发结节。完整尸检显示整个肝脏有离散的肿瘤结节,肿瘤过程未累及淋巴结或任何其他器官,无大体或显微镜下改变。肿瘤细胞表现出ALCL典型的组织学和免疫组化特征,对T细胞标志物CD-3和UCHL-1呈强免疫反应性。文献中仅报道过一例原发性肝脏ALCL,该肿瘤发生在免疫功能正常的患者中,对B或T细胞标志物无免疫反应性。据我们所知,本研究是首例报道的T细胞表型原发性肝脏间变性大细胞淋巴瘤病例。此外,这是首例在艾滋病患者中报道的原发性肝脏ALCL病例。

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