老年男性非cll型单克隆b细胞淋巴细胞增多症患者的唯一异常:21号单体。

Wilson Yeh, Dariusz Mrugala, Hannah Robinson, Carlos A Tirado
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摘要

目的:单克隆B细胞淋巴细胞增多症(MBL)是一种成熟B细胞少于5000个/μL的轻链限制性增殖,没有额外的临床或血液异常。对慢性淋巴细胞白血病(CLL)易感个体的同胞研究首先在健康人群中发现了单克隆B细胞,研究表明40岁以上人群中MBL患病率为3%至14%。非cll型MBL占所有MBL病例的不到20%,其进展不完全表征。我们报告一例85岁男性患者,其CD5-、CD19+、CD20亮型和lambda-限制性淋巴样细胞,其免疫表型结果提示为边缘区淋巴瘤(MZL)的前驱病变。核型分析显示,35个细胞中有3个为21单体,没有额外的细胞遗传学变化。在1984年至2003年间,仅在11例CLL病例中发现了21号单体作为CLL的唯一异常。作为脾脏和淋巴结边缘区淋巴瘤的唯一异常,在Mitelman肿瘤染色体畸变和基因融合数据库中仅记录了3例21号单体。21号单体作为肿瘤发生标志的意义尚不清楚。
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Monosomy 21, a Sole Abnormality in an Elderly Man with Non-CLL-Type Monoclonal B-cell Lymphocytosis.

Objectives: Monoclonal B-cell lymphocytosis (MBL) is a light-chain restricted proliferation of mature B cells fewer than 5000 cells/μL without additional clinical or hematologic abnormalities. Sibling studies of individuals genetically susceptible to chronic lymphocytic leukemia (CLL) first identified monoclonal B cells in otherwise healthy persons, and studies show a 3% to 14% prevalence for MBL in persons over 40 years of age. Non-CLL-type MBL accounts for less than 20% of all MBL cases, and its progression is incompletely characterized. Here we present the case of an 85-year-old man with CD5-, CD19+, CD20 bright, and lambda-restricted lymphoid cells whose immunophenotypic findings are suggestive for a precursor lesion to marginal zone lymphoma (MZL). Karyotyping showed monosomy 21 without additional cytogenetic changes in three of the 35 cells examined. Monosomy 21 as a sole abnormality in CLL has been detected in just 11 cases between 1984 and 2003. As a sole abnormality in splenic and nodal marginal zone lymphoma, only three instances of monosomy 21 have been recorded on the Mitelman Database of Chromosome Aberrations and Gene Fusions in Cancer. The significance of monosomy 21 as a marker for oncogenesis remains unclear.

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