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表达异常CD3和CD10并合并小淋巴细胞淋巴瘤的母细胞样变异型套细胞淋巴瘤:通过B细胞和T细胞受体基因重排建立克隆关系

Blastoid Variant Mantle Cell Lymphoma Expressing Aberrant CD3 and CD10 with Concurrent Small Lymphocytic Lymphoma: Establishment of a Clonal Relationship by B- and T-Cell Receptor Gene Rearrangements.

作者信息

Felten Christopher L, Chan Joel A, DeCastro Dulce R, Lopategui Jean, Rajurkar Swapnil P

机构信息

Gemini Diagnostics Hematopathology, 756 Lakefield Road Suite C, Westlake Village, CA 91361, USA.

San Antonio Regional Hospital, Department of Pathology, 999 San Bernardino Road, Upland, CA 91786, USA.

出版信息

Case Rep Hematol. 2018 Dec 5;2018:8303571. doi: 10.1155/2018/8303571. eCollection 2018.

Abstract

Mantle cell lymphoma (MCL) is an aggressive non-Hodgkin B-cell lymphoma typically expressing CD19, CD20, CD5, FMC-7, CyclinD1, and SOX-11 and harboring the IgH/CCND1 translocation. We report a blastoid variant of mantle cell lymphoma (MCL) involving an inguinal lymph node that, in addition to classical phenotypic and genetic findings, also aberrantly coexpresses surface CD10 and cytoplasmic CD3. Small lymphocytic lymphoma (SLL) was also present in the same lymph node and in the bone marrow. B- and T-cell gene rearrangement studies by PCR show the MCL and SLL to be clonally related. Expression of multiple aberrant antigens and concurrent lymphomas of different classifications can cause a diagnostic challenge. Awareness of such a presentation and integration of the data from morphologic evaluation, flow cytometry, immunohistochemistry, and FISH studies is required for proper diagnosis, prognosis, and therapy.

摘要

套细胞淋巴瘤(MCL)是一种侵袭性非霍奇金B细胞淋巴瘤,通常表达CD19、CD20、CD5、FMC-7、细胞周期蛋白D1和SOX-11,并存在IgH/CCND1易位。我们报告了一例累及腹股沟淋巴结的母细胞样套细胞淋巴瘤(MCL),除了典型的表型和基因特征外,还异常共表达表面CD10和细胞质CD3。同一淋巴结和骨髓中还存在小淋巴细胞淋巴瘤(SLL)。通过聚合酶链反应进行的B细胞和T细胞基因重排研究显示,MCL和SLL存在克隆相关性。多种异常抗原的表达以及不同分类的并发淋巴瘤可能会带来诊断挑战。正确的诊断、预后评估和治疗需要认识到这种表现,并整合形态学评估、流式细胞术、免疫组织化学和荧光原位杂交研究的数据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6926/6305046/fc879edf1d5f/CRIHEM2018-8303571.001.jpg

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