Zen Paulo R G, Capra Marcelo E Z, Silla Lúcia M R, Loss Jiseh F, Fernandes Mário S, Jacques Sidia M C, Paskulin Giorgio A
Clinical Genetics and Post-Graduation Program in Pathology, Federal University of Health Sciences of Porto Alegre, Rua Sarmento Leite 245/403, Porto Alegre, 90050-170, RS, Brazil.
Cancer Genet Cytogenet. 2009 Jan 15;188(2):112-7. doi: 10.1016/j.cancergencyto.2008.09.005.
A Brazilian sample of 58 patients with acute lymphoblastic leukemia has been prospectively followed up with the objective of evaluating evolution of disease. Age ranged from 6 months to 16 years. Of the 58 patients, 11 were positive and 47 were negative for the ETV6/RUNX1 fusion (previously known as TEL/AML1). After a minimum follow-up period of 57 months, 2 of the ETV6/RUNX1(+) patients had died and 11 of the ETV6/RUNX1(-), for an overall survival of 77.6%. Among the 11 ETV6/RUNX1(+) patients (age range, 2 years to 13 years 7 months), all achieved a complete remission; the average overall survival was 64.2 months and the average event-free survival was 61.7 months. Among the 47 ETV6/RUNX1(-) patients, 4 did not have a complete remission; the average overall survival was of 60.8 months and the average event-free survival was 57.2 months. No significant difference was observed between overall survival and event-free survival, nor in any of the other data comparatively analyzed. The patients had a cure rate similar to that described in literature. In this small sample population, the presence of the ETV6/RUNX1 fusion did not identify statistical difference in prognosis.
对58例急性淋巴细胞白血病巴西患者样本进行了前瞻性随访,目的是评估疾病进展情况。年龄范围为6个月至16岁。在这58例患者中,11例ETV6/RUNX1融合基因(以前称为TEL/AML1)检测呈阳性,47例呈阴性。经过至少57个月的随访,11例ETV6/RUNX1(+)患者中有2例死亡,47例ETV6/RUNX1(-)患者中有11例死亡,总生存率为77.6%。在11例ETV6/RUNX1(+)患者(年龄范围为2岁至13岁7个月)中,所有患者均实现完全缓解;平均总生存期为64.2个月,平均无事件生存期为61.7个月。在47例ETV6/RUNX1(-)患者中,4例未实现完全缓解;平均总生存期为60.8个月,平均无事件生存期为57.2个月。总生存期和无事件生存期之间未观察到显著差异,在任何其他比较分析的数据中也未观察到显著差异。这些患者的治愈率与文献中描述的相似。在这个小样本群体中,ETV6/RUNX1融合基因的存在并未显示出预后的统计学差异。