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伴有CCND2基因与IGL基因座易位的CD5阳性、细胞周期蛋白D1阴性套细胞淋巴瘤

CD5-positive, cyclinD1-negative mantle cell lymphoma with a translocation involving the CCND2 gene and the IGL locus.

作者信息

Shiller Shirley Michelle, Zieske Arthur, Holmes Houston, Feldman Andrew L, Law Mark E, Saad Rana

机构信息

Department of Pathology, Baylor University Medical Center, Dallas, TX, USA.

出版信息

Cancer Genet. 2011 Mar;204(3):162-4. doi: 10.1016/j.cancergencyto.2010.08.016.

DOI:10.1016/j.cancergencyto.2010.08.016
PMID:21504716
Abstract

Distinguishing mantle cell lymphoma (MCL), from low-grade B-cell lymphoma is important because MCL is clinically more aggressive and is treated differently. Though most MCL overexpress cyclinD1 (CCND1) and have a t(11;14)(q13;q32), MCL that are negative for CCND1 exist. Some have translocations involving cyclinD2 (CCND2) and either the immunoglobulin heavy chain or kappa light chain locus. We present a CD5-positive, CCND1-negative B-cell lymphoma with a novel translocation involving CCND2 and the immunoglobulin lambda (IGL) gene. A 64-year-old male underwent resection of a polypoid mass of the ileum. Histology showed atypical, medium-sized lymphoid cells positive for CD20, CD5, CD43, and CCND2 by immunohistochemistry, and negative for CCND1, CCND3, and p27. Fluorescence in situ hybridization was negative for CCND1 abnormalities, but demonstrated a CCND2/IGL fusion. Clinical workup revealed stage IV disease. Current diagnostic criteria are insufficient for subclassifying this case, highlighting the need for additional studies on CCND2-translocated B-cell lymphomas to guide therapy appropriately.

摘要

区分套细胞淋巴瘤(MCL)与低度B细胞淋巴瘤很重要,因为MCL在临床上侵袭性更强,治疗方式也不同。尽管大多数MCL过度表达细胞周期蛋白D1(CCND1)且具有t(11;14)(q13;q32),但也存在CCND1阴性的MCL。一些MCL存在涉及细胞周期蛋白D2(CCND2)以及免疫球蛋白重链或κ轻链基因座的易位。我们报告一例CD5阳性、CCND1阴性的B细胞淋巴瘤,其具有涉及CCND2和免疫球蛋白λ(IGL)基因的新型易位。一名64岁男性接受了回肠息肉样肿物切除术。组织学检查显示,免疫组化结果显示非典型中等大小淋巴细胞CD20、CD5、CD43和CCND2呈阳性,CCND1、CCND3和p27呈阴性。荧光原位杂交显示CCND1无异常,但存在CCND2/IGL融合。临床检查显示为IV期疾病。目前的诊断标准不足以对该病例进行亚分类,这凸显了对CCND2易位B细胞淋巴瘤进行更多研究以适当指导治疗的必要性。

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