Department of Leukemia, University of Texas MD Anderson Cancer Center, Houston, Texas 77030, USA.
Am J Hematol. 2012 Mar;87(3):317-8. doi: 10.1002/ajh.22258. Epub 2012 Jan 7.
A subgroup of patients with core binding factor acute myeloid leukemias (AML) is characterized by the presence of the fusion gene CBFb-Myh11. At the cytogenetic level, most of these patients are identified by the presence of an inversion of chromosome 16 [inv(16)(p13q22)] and rarely by a translocation t(16;16)(p13;q22). The aim of this study is to describe the natural history of patients with t(16;16) [N = 6] treated at MD Anderson Cancer Center and compared them with a cohort of patients with inv(16)(p13q22) [n = 61]. In patients with t(16;16) the complete remission rate (CR) was 100% when treated with a combination of fludarabine and high-dose cytarabine. Median overall survival (OS) had not been achieved. There was no difference in response or OS or progression free survival between both groups. Presence of additional chromosomal abnormalities and molecular aberrations had no effect on prognosis. In conclusion, and consistent with previous reports, the natural history of patients with t(16:16)(p13;q22) is similar to that of classic patients with inv16 AML and therefore should be treated similarly.
伴有 CBFb-Myh11 融合基因的急性髓系白血病(AML)亚组患者的特征为存在染色体 16 的倒位 [inv(16)(p13q22)],大多数患者可通过这种细胞遗传学改变来识别,而很少通过 t(16;16)(p13;q22)易位来识别。本研究旨在描述在 MD 安德森癌症中心接受治疗的伴有 t(16;16) [N = 6]的患者的自然病史,并将其与伴有 inv(16)(p13q22) [n = 61]的患者队列进行比较。伴有 t(16;16)的患者经氟达拉滨联合高剂量阿糖胞苷治疗后完全缓解率(CR)为 100%。中位总生存期(OS)尚未达到。两组之间在反应、OS 或无进展生存期方面没有差异。存在其他染色体异常和分子异常对预后没有影响。总之,与之前的报道一致,伴有 t(16:16)(p13;q22)的患者的自然病史与经典的伴有 inv16 AML 患者的自然病史相似,因此应进行类似的治疗。