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毛细胞的病理生理学

The pathophysiology of the hairy cell.

作者信息

Cawley J C

机构信息

Department of Haematology, University of Liverpool, Third Floor Duncan Building, Daulby Street, Liverpool L69 3GA, UK.

出版信息

Hematol Oncol Clin North Am. 2006 Oct;20(5):1011-21. doi: 10.1016/j.hoc.2006.06.002.

DOI:10.1016/j.hoc.2006.06.002
PMID:16990104
Abstract

HCs are clonal late B cells that are related to memory cells and display specific features of activation. Many of the distinctive features of HCs (eg, morphology, TRAP) are related to this specific activation. Many of the distinctive histologic features of HCL can be related to constitutive production of cytokines (eg, FGF, fibrosis) and to the expression/activation of adhesion receptors (eg, alpha(4)beta(1), alpha(5)beta(1) and alpha(v)beta(3) integrins, CD44v3). HCs usually have mutated IGVH genes and have no consistent or specific chromosome abnormalities (5q additions and 7q deletions in a minority). The signals that are responsible for several of the phenotypic features of HCs have been identified, but the nature of the underlying oncogenic events remains unknown.

摘要

毛细胞(HCs)是与记忆细胞相关的克隆性晚期B细胞,并表现出特定的激活特征。HCs的许多独特特征(如形态、抗酒石酸酸性磷酸酶)都与这种特定的激活有关。毛细胞白血病(HCL)的许多独特组织学特征可能与细胞因子的组成性产生(如成纤维细胞生长因子、纤维化)以及黏附受体的表达/激活(如α(4)β(1)、α(5)β(1)和α(v)β(3)整合素、CD44v3)有关。HCs通常具有免疫球蛋白重链可变区(IGVH)基因突变,且没有一致或特定的染色体异常(少数有5号染色体长臂增加和7号染色体长臂缺失)。虽然已经确定了导致HCs某些表型特征的信号,但潜在致癌事件的本质仍然未知。

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The pathophysiology of the hairy cell.毛细胞的病理生理学
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