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以神经受累为首发临床表现的慢性淋巴细胞白血病:一种罕见现象及对更好预测标志物的需求

Chronic lymphocytic leukemia with clinical debut as neurological involvement: a rare phenomenon and the need for better predictive markers.

作者信息

Rojas-Hernandez Cristhiam M, Nemunaitis Jacklyn, Marjon Kristopher D, Bustamante Daniel, Zhang Qian-Yun, Gillette Jennifer M

机构信息

Division of Hematology and Oncology, University of New Mexico Health Sciences Center, Albuquerque, NM USA.

Department of Medicine, University of New Mexico Health Sciences Center, Albuquerque, NM USA.

出版信息

BMC Hematol. 2017 Feb 2;17:3. doi: 10.1186/s12878-017-0073-0. eCollection 2017.

DOI:10.1186/s12878-017-0073-0
PMID:28174663
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5290634/
Abstract

BACKGROUND

Chronic lymphocytic leukemia (CLL) is the most common leukemia in Western countries. The frequency of symptomatic central nervous system (CNS) involvement is unknown but thought to be a rare phenomenon. Currently there are no known risk factors for CNS involvement.

CASE PRESENTATION

We describe a clinically staged low-risk CLL case that presented with symptomatic CNS involvement and progressed rapidly to death. Evaluation of the surface adhesion molecules identified a markedly altered expression pattern of the integrin, CD49d, and the tetraspanin, CD82, in the index case when compared to similar low-risk CLL cases. We found that the early Rai clinical stage CLL patients showed linear correlation for the co-expression of CD82 and CD49d. In contrast, this unique index case with CNS involvement, which has the same Rai clinical stage, had a significantly lower expression of CD82 and higher expression of CD49d.

CONCLUSIONS

These data suggest that the expression profile of CD49d and CD82 may represent potential biomarkers for patients with increased propensity of CNS involvement. Moreover, this study illustrates the critical need for a better mechanistic understanding of how specific adhesion proteins regulate the interactions between CLL cells and various tissue sites.

摘要

背景

慢性淋巴细胞白血病(CLL)是西方国家最常见的白血病。有症状的中枢神经系统(CNS)受累频率尚不清楚,但被认为是一种罕见现象。目前尚无已知的CNS受累危险因素。

病例介绍

我们描述了一例临床分期为低风险的CLL病例,该病例出现有症状的CNS受累并迅速进展至死亡。与类似的低风险CLL病例相比,对表面粘附分子的评估发现该索引病例中整合素CD49d和四跨膜蛋白CD82的表达模式明显改变。我们发现早期Rai临床分期的CLL患者中CD82和CD49d的共表达呈线性相关。相比之下,这例具有相同Rai临床分期且有CNS受累的独特索引病例,其CD82表达显著降低而CD49d表达升高。

结论

这些数据表明,CD49d和CD82的表达谱可能代表CNS受累倾向增加患者的潜在生物标志物。此外,本研究表明迫切需要更好地从机制上理解特定粘附蛋白如何调节CLL细胞与各种组织部位之间的相互作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5865/5290634/852a52e8bc8f/12878_2017_73_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5865/5290634/852a52e8bc8f/12878_2017_73_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5865/5290634/852a52e8bc8f/12878_2017_73_Fig1_HTML.jpg

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