Gerbing Robert B, Alonzo Todd A, Sung Lillian, Gamis Alan S, Meshinchi Soheil, Plon Sharon E, Bertuch Alison A, Gramatges Maria M
Robert B. Gerbing and Todd A. Alonzo, Children's Oncology Group, Monrovia; Todd A. Alonzo, University of Southern California, Los Angeles, CA; Lillian Sung, Hospital for Sick Children, Toronto, Ontario, Canada; Alan S. Gamis, Children's Mercy Hospitals and Clinics, Kansas City, MO; Soheil Meshinchi, University of Washington School of Medicine, Seattle, WA; and Sharon E. Plon, Alison A. Bertuch, and Maria M. Gramatges, Baylor College of Medicine, Houston, TX.
J Clin Oncol. 2016 Nov 1;34(31):3766-3772. doi: 10.1200/JCO.2016.66.9622.
Purpose Suboptimal outcomes for children with acute myeloid leukemia (AML) necessitate maximally intensive therapy. Consequently, serious adverse events, such as prolonged periods of profound myelosuppression, contribute to AML treatment-related mortality. Telomeres, the repetitive DNA-protein structures at chromosome ends, influence cellular replicative capacity in that critically short telomeres can induce cell senescence or apoptosis. Our objective was to evaluate the impact of telomere length on duration of post-therapy neutropenia in a pediatric AML cohort. Patients and Methods Patients were diagnosed with de novo AML, enrolled in Children's Oncology Group study AAML0531, and included those with (n = 53) and without (n = 62) significantly delayed neutrophil recovery after chemotherapy. We used quantitative polymerase chain reaction to measure telomere content (TC), a validated proxy for telomere length, from remission bone marrow samples obtained after the second induction chemotherapy course. Results Less TC was significantly associated with prolonged neutropenia after the fourth ( P < .001) and fifth chemotherapy courses ( P = .002). Cox regression adjusting for age at diagnosis confirmed that TC remained independently predictive of time to recovery of absolute neutrophil count for both the fourth and fifth courses ( P = .002 and .009, respectively). DNA from patients was analyzed for germline mutations in four telomere maintenance genes associated with telomere biology disorders. Sequence analysis revealed no enrichment of rare or novel variants in the delayed recovery group. Conclusion Our results suggest that TC at end of AML induction is associated with hematopoietic reconstitution capacity independently of age and may identify those at highest risk for markedly delayed bone marrow recovery after AML therapy.
目的 急性髓系白血病(AML)患儿的预后欠佳,需要接受最大强度的治疗。因此,诸如长时间严重骨髓抑制等严重不良事件会导致AML治疗相关死亡率上升。端粒是染色体末端的重复性DNA-蛋白质结构,会影响细胞的复制能力,因为极短的端粒会诱导细胞衰老或凋亡。我们的目的是评估端粒长度对儿科AML队列中治疗后中性粒细胞减少持续时间的影响。
患者与方法 患者被诊断为新发AML,参加了儿童肿瘤学组研究AAML0531,包括化疗后中性粒细胞恢复显著延迟的患者(n = 53)和未延迟的患者(n = 62)。我们使用定量聚合酶链反应来测量端粒含量(TC),这是一种经过验证的端粒长度替代指标,测量对象为第二次诱导化疗疗程后获得的缓解期骨髓样本。
结果 较低的TC与第四次(P < .001)和第五次化疗疗程后中性粒细胞减少时间延长显著相关(P = .002)。对诊断时年龄进行校正的Cox回归分析证实,TC仍然独立预测第四次和第五次疗程中绝对中性粒细胞计数恢复的时间(分别为P = .002和P = .009)。对患者的DNA进行分析,以检测与端粒生物学障碍相关的四个端粒维持基因中的种系突变。序列分析显示,延迟恢复组中未富集罕见或新变体。
结论 我们的结果表明,AML诱导结束时的TC与造血重建能力相关,与年龄无关,并且可能识别出AML治疗后骨髓恢复明显延迟风险最高的患者。