Laboratory of Cytogenetics, ICBAS-Institute of Biomedical Sciences Abel Salazar, UP, Porto, Portugal.
Cell Prolif. 2010 Dec;43(6):573-8. doi: 10.1111/j.1365-2184.2010.00706.x.
Fanconi anaemia (FA) is a cancer-prone chromosome instability syndrome characterized by hypersensitivity to DNA cross-linking agents, such as diepoxybutane (DEB). Previous studies have shown that normal red blood cells (RBC) can protect cultured lymphocytes against chromosomal breaks induced by DEB. The present study was designed to analyse influence of RBCs from normal individuals on frequency of DEB-induced chromosome breaks in lymphocyte cultures from FA patients.
A comparative study was performed between DEB-induced chromosome breaks in cultures of FA lymphocytes with either autologous or heterologous RBCs. A further comparative study was carried out between whole blood cultures from FA patients performed on two occasions, before and 1 week after transfusion of RBCs.
It was observed that normal RBCs compared to FA RBCs, partially reduced chromosome breaks in cultured FA lymphocytes. A significant reduction in DEB-induced breaks was also observed in FA cultured lymphocytes obtained 1 week after transfusion of RBCs, in comparison to those observed in the same patients before RBC transfusion.
This study shows that DEB-induced chromosome instability in FA lymphocytes is partially reduced by normal RBCs. This effect may have some clinical relevance in vivo, whenever FA patients receive a RBC transfusion.
范可尼贫血(FA)是一种易患癌症的染色体不稳定综合征,其特征是对 DNA 交联剂(如二环氧丁烷(DEB))高度敏感。先前的研究表明,正常的红细胞(RBC)可以保护培养的淋巴细胞免受 DEB 诱导的染色体断裂。本研究旨在分析正常个体的 RBC 对 FA 患者淋巴细胞培养中 DEB 诱导的染色体断裂频率的影响。
对 FA 淋巴细胞培养中 DEB 诱导的染色体断裂进行了自体或异体 RBC 的比较研究。还对 FA 患者全血培养进行了进一步的比较研究,在输血前和输血后 1 周进行。
观察到与 FA RBC 相比,正常 RBC 部分减少了培养的 FA 淋巴细胞中的染色体断裂。与 FA 患者输血前相比,输血后 1 周获得的 FA 培养淋巴细胞中,DEB 诱导的断裂也明显减少。
本研究表明,正常 RBC 部分减少了 FA 淋巴细胞中 DEB 诱导的染色体不稳定性。在 FA 患者接受 RBC 输血时,这种作用在体内可能具有一定的临床意义。