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滤泡性淋巴瘤的生物学与治疗。

Biology and treatment of follicular lymphoma.

机构信息

Molecular Pathology Laboratory, Hematopathology Unit, Department of Hematology and Oncological Sciences L and A Seràgnoli, S Orsola Malpighi Hospital, University of Bologna, Via Massarenti 9, Bologna, Italy.

出版信息

Expert Rev Hematol. 2009 Oct;2(5):533-47. doi: 10.1586/ehm.09.49.

DOI:10.1586/ehm.09.49
PMID:21083019
Abstract

Follicular lymphoma (FL) is the second most common lymphoid tumor. It is composed of elements resembling those of normal germinal centers. In particular, it is constituted by small centrocytes and large centroblasts, typically CD10+, CD19+, CD20+, CD79a+ and BCL6+, with follicular growth pattern. The molecular hallmark of FL is the t(14;18)(q32;q21) translocation, which leads to inappropriate BCL2 expression. This feature, other than representing a pathogenetic primary event, constitutes a suitable diagnostic marker, as well as a target for minimal residual disease monitoring and, hopefully, future therapies. Clinically, FL presents with indolent behavior, characterized by prompt response to initial therapy but almost invariably subsequent relapses. Novel approaches, including stem cell transplantation, monoclonal antibodies and innovative agents, should be then considered for improving long-term results.

摘要

滤泡性淋巴瘤(FL)是第二常见的淋巴肿瘤。它由类似于正常生发中心的成分组成。特别是,它由小中心细胞和大中心母细胞组成,通常为 CD10+、CD19+、CD20+、CD79a+ 和 BCL6+,具有滤泡生长模式。FL 的分子特征是 t(14;18)(q32;q21)易位,导致 BCL2 表达异常。除了代表一种发病的主要事件外,这一特征还构成了一个合适的诊断标志物,以及一个用于微小残留疾病监测的靶点,并有望成为未来治疗的靶点。临床上,FL 表现为惰性行为,其特征是对初始治疗有迅速反应,但几乎总是随后复发。然后应该考虑采用新的方法,包括干细胞移植、单克隆抗体和创新药物,以改善长期结果。

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