Dos Santos Patricia, Panero Julieta, Palau Nagore Virginia, Stanganelli Carmen, Bezares Raimundo F, Slavutsky Irma
Laboratorio de Genética de Neoplasias Linfoides, Instituto de Medicina Experimental, CONICET-Academia Nacional de Medicina, J.A. Pacheco de Melo 3081, C1425AUM, Buenos Aires, Argentina.
División Patología Molecular, Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina.
Tumour Biol. 2015 Nov;36(11):8317-24. doi: 10.1007/s13277-015-3556-2. Epub 2015 May 26.
Telomeric dysfunction has been proposed as an emerging prognostic factor in chronic lymphocytic leukemia (CLL). We have explored the relationship between telomere length (TL) and chromosome alterations studied by fluorescence in situ hybridization (FISH) and conventional cytogenetics in 107 newly diagnosed CLL patients; 61 normal controls were also evaluated. Results were correlated with clinical parameters and outcome. Absolute TL measurement was carried out on DNA samples by real-time quantitative PCR. A significant telomere shortening in patients compared to controls was observed (p = 0.0001). The analysis taking into account FISH risk groups showed shorter TLs in cases with del11q/17p compared to patients with 13q14 deletion as a single alteration (p = 0.0037), no alterations (NA) (p = 0.028), and cases with abnormal karyotypes (p = 0.014). In addition, a significant TL reduction in cases with two or more anomalies with respect to those with NA (p = 0.033) and with one alteration (p = 0.045), and no differences compared to cases with deletions 11q/17p were observed. Patients with only one anomaly did not show statistical differences with respect to controls; meanwhile, a significant TL reduction in cases with two or more aberrations was observed (p = 0.025). The shortest telomeres were associated to 11q/17p deletion with significant differences compared to the remaining groups (p ≤ 0.045). Significantly shorter treatment free survival in patients with two or more alterations compared to those with NA plus one abnormality was observed (p = 0.0006). Our findings support the association between short TL and chromosome alterations in CLL and indicate the importance of telomere dysfunction in driving genomic instability in this pathology.
端粒功能障碍已被提出作为慢性淋巴细胞白血病(CLL)一种新出现的预后因素。我们探讨了107例新诊断CLL患者的端粒长度(TL)与通过荧光原位杂交(FISH)和传统细胞遗传学研究的染色体改变之间的关系;还评估了61名正常对照。结果与临床参数和预后相关。通过实时定量PCR对DNA样本进行绝对TL测量。观察到患者的端粒明显短于对照(p = 0.0001)。考虑FISH风险组的分析显示,与仅存在13q14缺失的患者相比,del11q/17p患者的TL更短(p = 0.0037),无改变(NA)的患者(p = 0.028)以及核型异常的患者(p = 0.014)。此外,与NA患者(p = 0.033)和存在一种改变的患者(p = 0.045)相比,存在两种或更多异常的患者的TL显著降低,与del11q/17p缺失的患者相比未观察到差异。仅存在一种异常的患者与对照相比未显示统计学差异;同时,观察到存在两种或更多畸变的患者的TL显著降低(p = 0.025)。最短的端粒与11q/17p缺失相关,与其余组相比有显著差异(p≤0.045)。观察到与NA加一种异常的患者相比,存在两种或更多改变的患者的无治疗生存期显著缩短(p = 0.0006)。我们的研究结果支持CLL中短TL与染色体改变之间的关联,并表明端粒功能障碍在驱动这种病理状态下的基因组不稳定中的重要性。