Clinical Pathology Department, Ain Shams University, Cairo, Egypt.
Eur J Haematol. 2012 Feb;88(2):118-27. doi: 10.1111/j.1600-0609.2011.01734.x. Epub 2012 Jan 4.
Although BIRC6/Apollon seems to play a critical role as an antiapoptotic regulator, its clinical relevance in acute leukemia remains largely elusive. Therefore, we aimed to investigate BIRC6 gene expression in childhood acute leukemia in relation to clinicopathological characteristics at presentation, therapeutic response, and prognosis.
BIRC6 expression level was assessed in 75 children with acute leukemia; 30 patients with acute myeloblastic leukemia (AML) and 45 patients with acute lymphoblastic leukemia (ALL) using real-time quantitative reverse transcriptase-polymerase chain reaction.
The median level of BIRC6 expression did not differ significantly between AML and ALL patients. BIRC6 expression level was higher in patients with AML and ALL with extramedullary involvement, white blood cell (WBC) count ≥ 10 × 10(9) /L, and unfavorable cytogenetics at diagnosis. BIRC6 gene expression was higher in patients with unfavorable response to therapy at day 14, those who developed relapse or died in both leukemic groups. The best cutoff value of BIRC6 to predict therapeutic response and disease outcome was determined. AML and ALL patients with BIRC6 overexpression had significantly shorter overall and disease free survivals.
This is the first report to study BIRC6 gene in pediatric ALL. Our results suggested that BIRC6 gene expression could be considered as an adverse risk factor in childhood acute leukemia and, hence, could be used to guide therapeutic regimens.
尽管 BIRC6/Apollon 似乎作为一种抗凋亡调节剂发挥着关键作用,但它在急性白血病中的临床相关性仍很大程度上难以捉摸。因此,我们旨在研究 BIRC6 基因在儿童急性白血病中的表达与发病时的临床病理特征、治疗反应和预后的关系。
使用实时定量逆转录聚合酶链反应(qRT-PCR)评估 75 例急性白血病儿童(30 例急性髓细胞白血病(AML)和 45 例急性淋巴细胞白血病(ALL))的 BIRC6 表达水平。
AML 和 ALL 患者之间 BIRC6 表达水平的中位数无显著差异。BIRC6 表达水平在有髓外浸润、白细胞计数≥10×10(9)/L 和诊断时不良细胞遗传学的 AML 和 ALL 患者中较高。在治疗第 14 天治疗反应不良、在两种白血病组中均发生复发或死亡的患者中,BIRC6 基因表达更高。确定了预测治疗反应和疾病结局的最佳 BIRC6 截断值。BIRC6 过表达的 AML 和 ALL 患者的总生存期和无病生存期明显缩短。
这是首次在儿科 ALL 中研究 BIRC6 基因的报告。我们的研究结果表明,BIRC6 基因表达可被视为儿童急性白血病的不良风险因素,因此可用于指导治疗方案。