Pepper Chris, Lin Thet Thet, Pratt Guy, Hewamana Saman, Brennan Paul, Hiller Louise, Hills Robert, Ward Rachel, Starczynski Jane, Austen Belinda, Hooper Laura, Stankovic Tatjana, Fegan Chris
Department of Haematology, School of Medicine, Cardiff University, Cardiff, United Kingdom.
Blood. 2008 Nov 1;112(9):3807-17. doi: 10.1182/blood-2008-05-157131. Epub 2008 Jul 3.
Bcl-2 family proteins play a critical role in the regulation of apoptosis in chronic lymphocytic leukemia (CLL). However, their association with established prognostic markers is unknown. In this study, we analyzed the expression of Bcl-2, Bax, and Mcl-1 in 185 CLL patients and evaluated their relationship with other prognostic markers, in vitro sensitivity to fludarabine, and clinical outcome. Mcl-1 expression was significantly correlated with stage of disease (P < .001), lymphocyte doubling time (P = .01), V(H) gene mutation status (P < .001), CD38 expression (P < .001), and ZAP-70 expression (P = .003). In addition, Mcl-1 and Mcl-1/Bax ratios showed strong correlations with in vitro resistance to fludarabine (P = .005 and P < .001, respectively). Furthermore, elevated Mcl-1 expression and Mcl-1/Bax ratios were predictive of time to first treatment in the whole cohort (P < .001 and P < .001, respectively) and in stage A patients only (P = .002 and P = .001, respectively). Taken together, our data show that Mcl-1 is a key controller of in vitro drug resistance and is an important regulator of disease progression and outcome in CLL. It therefore represents a promising therapeutic target in this incurable condition. The close correlation between Mcl-1 expression and V(H) gene mutation status, CD38 expression, and ZAP-70 expression offers a biologic explanation for their association with adverse prognosis.
Bcl-2家族蛋白在慢性淋巴细胞白血病(CLL)的细胞凋亡调控中起关键作用。然而,它们与已确立的预后标志物之间的关联尚不清楚。在本研究中,我们分析了185例CLL患者中Bcl-2、Bax和Mcl-1的表达,并评估了它们与其他预后标志物、对氟达拉滨的体外敏感性以及临床结局的关系。Mcl-1表达与疾病分期(P <.001)、淋巴细胞倍增时间(P =.01)、V(H)基因突变状态(P <.001)、CD38表达(P <.001)和ZAP-70表达(P =.003)显著相关。此外,Mcl-1及Mcl-1/Bax比值与对氟达拉滨的体外耐药性密切相关(分别为P =.005和P <.001)。此外,Mcl-1表达升高及Mcl-1/Bax比值升高可预测整个队列(分别为P <.001和P <.001)以及仅A期患者(分别为P =.002和P =.001)的首次治疗时间。综上所述,我们的数据表明,Mcl-1是体外耐药性的关键调控因子,是CLL疾病进展和结局的重要调节因子。因此,它是这种无法治愈疾病中有前景的治疗靶点。Mcl-1表达与V(H)基因突变状态、CD38表达和ZAP-70表达之间的密切相关性为它们与不良预后的关联提供了生物学解释。