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伴有继发性TP53缺失的非淋巴结性CD5阴性套细胞淋巴瘤

Non-Nodal CD5-Negative Mantle Cell Lymphoma with Secondary TP53 Deletion.

作者信息

McCall Robert K, Pang Changlee S, Pettenati Mark J

机构信息

Molecular Pathology Laboratory Network, Maryville, TN, USA.

University of Colorado School of Medicine, Aurora, CO, USA.

出版信息

Case Rep Hematol. 2020 Mar 18;2020:9185432. doi: 10.1155/2020/9185432. eCollection 2020.

Abstract

Mantle cell lymphoma is a non-Hodgkin lymphoproliferative neoplasm with several clinical and morphologic variants linked, primarily, through genetic derangement of the locus. Aberrant phenotypes have been described, though prognostic data in such cohorts are limited due to a paucity of cases. We report a case of mantle cell lymphoma with non-nodal clinical presentation, aberrant loss of CD5 expression, and concomitant cytogenetic deletion of 17p. While non-nodal disease is often associated with an improved prognosis in mantle cell lymphoma, this 67-year-old patient experienced a more challenging clinical course with a poor initial response to chemotherapy. Therefore, this case may represent a type of non-nodal mantle cell lymphoma with a prognosis similar to that of classical cases due to the additional phenotypic and genetic alterations found in this patient.

摘要

套细胞淋巴瘤是一种非霍奇金淋巴瘤增殖性肿瘤,有几种临床和形态学变异型,主要通过该基因座的基因紊乱联系在一起。尽管已经描述了异常表型,但由于病例稀少,此类队列中的预后数据有限。我们报告一例套细胞淋巴瘤,其临床表现为非淋巴结型,CD5表达异常缺失,同时伴有17p细胞遗传学缺失。虽然非淋巴结疾病在套细胞淋巴瘤中通常与较好的预后相关,但这位67岁的患者临床病程更具挑战性,对化疗的初始反应较差。因此,由于该患者存在额外的表型和基因改变,此病例可能代表一种预后与经典病例相似的非淋巴结型套细胞淋巴瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/786c/7106885/55d2ec9fd1c4/CRIHEM2020-9185432.001.jpg

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