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伴有CD10异常表达的套细胞淋巴瘤。

Mantle cell lymphoma with aberrant expression of CD10.

作者信息

Zanetto U, Dong H, Huang Y, Zhang K, Narbaitz M, Sapia S, Kostopoulos I, Liu H, Du M-Q, Bacon C M

机构信息

Department of Pathology, City Hospital, Birmingham, UK.

出版信息

Histopathology. 2008 Jul;53(1):20-9. doi: 10.1111/j.1365-2559.2008.03060.x. Epub 2008 Jun 2.

Abstract

AIMS

Morphological, immunophenotypic and genetic heterogeneity amongst mantle cell lymphomas (MCLs) can lead to difficulties in diagnosis and management. The aim was to describe the clinical and pathological features of MCLs with aberrant expression of CD10.

METHODS AND RESULTS

Of 17 specimens from 13 patients, 14 expressed CD10 and three (presenting before or after a CD10+ specimen) did not. All expressed cyclin D1 and carried the t(11;14)(q13;q32)/CCND1-IGH translocation. Similar to non-selected MCL patients, most patients had disseminated disease and an adverse clinical course. Five specimens showed pleomorphic blastoid morphology and blastoid transformation was associated with a change in phenotype, including gain or loss of CD10. Additional phenotypic variations likely to cause diagnostic difficulty were present in eight specimens: five were CD5- and five (all CD10+) expressed Bcl-6. One Bcl-6+ case carried a BCL-6 translocation and three others had extra copies of the BCL-6 gene. Sequence analysis of the immunoglobulin heavy chain variable region in five cases showed only one to have low-level somatic mutation, indicating that they did not arise from germinal centre B cells.

CONCLUSIONS

Expression of CD10 by MCL is often associated with other variant morphological, immunophenotypic or genetic features, but does not reflect derivation from germinal centre B cells.

摘要

目的

套细胞淋巴瘤(MCL)的形态学、免疫表型和基因异质性可能导致诊断和管理困难。本研究旨在描述CD10表达异常的MCL的临床和病理特征。

方法与结果

13例患者的17份标本中,14份表达CD10,3份(在CD10阳性标本之前或之后出现)不表达。所有标本均表达细胞周期蛋白D1,并携带t(11;14)(q13;q32)/CCND1-IGH易位。与未选择的MCL患者相似,大多数患者有播散性疾病且临床病程不良。5份标本显示多形性母细胞样形态,母细胞样转化与表型改变有关,包括CD10的获得或缺失。8份标本存在可能导致诊断困难的其他表型变异:5份CD5阴性,5份(均为CD10阳性)表达Bcl-6。1例Bcl-6阳性病例携带BCL-6易位,另外3例有额外的BCL-6基因拷贝。5例免疫球蛋白重链可变区序列分析显示只有1例有低水平体细胞突变,表明它们并非起源于生发中心B细胞。

结论

MCL中CD10的表达常与其他形态学、免疫表型或基因变异特征相关,但并不反映其起源于生发中心B细胞。

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