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[急性髓系白血病中CEBPA突变类型及其对预后的影响]

[Type of CEBPA mutations in acute myeloid leukemia and their effect on prognosis].

作者信息

Mao Y Y, Cai H, Cao X X, Feng J, Zhang L, Zhou D B, Li J

机构信息

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2024 Jun 14;45(6):556-560. doi: 10.3760/cma.j.cn121090-20240316-00098.

Abstract

To demonstrate the type of CEBPA gene mutations among patients with acute myeloid leukemia (AML), clinical characteristics, and prognostic effect on patient outcomes. Demographic data, clinical features, laboratory characteristics, and data about treatment and follow-up of 57 patients with CEBPA mutated AML diagnosed at Peking Union Medical College Hospital between April 2016 and November 2022 were collected and analyzed. In total, 57 patients with CEBPA mutation accounted for 16.1% of all the 353 patients with AML, among which 28 patients had CEBPA-bZIPinf and 29 had CEBPA-other. Compared with the CEBPA-other group, the CEBPA-bZIPinf group was younger (54 64 years, =0.010), de novo AML was more common (=0.001), and the level of bone marrow blast was higher (68.0% 36.3%, =0.001). Moreover, 24 patients from the CEBPA-bZIPinf group and 19 from the CEBPA-other group received chemotherapy. The one-course complete remission (CR) rate of the CEBPA-bZIPinf group was significantly higher than that of the CEBPA-other (87.5% 47.4%, =0.010) and CEBPA-wt (87.5% 50.3%, =0.002) groups. After a median follow-up of 11 months, the median OS of the CEBPA-bZIPinf group was significantly longer than that of the CEBPA-wt group (not reached 22.1 months, =0.012) . CEBPA-bZIPinf mutated AML is a unique clinical entity, with a younger age of diagnosis, better response to chemotherapy, and better prognosis.

摘要

为了阐明急性髓系白血病(AML)患者中CEBPA基因突变的类型、临床特征及其对患者预后的影响,我们收集并分析了2016年4月至2022年11月在北京协和医院确诊的57例CEBPA基因突变型AML患者的人口统计学数据、临床特征、实验室检查结果以及治疗和随访数据。57例CEBPA基因突变患者占全部353例AML患者的16.1%,其中28例为CEBPA-bZIPinf突变型,29例为CEBPA-其他突变型。与CEBPA-其他突变型组相比,CEBPA-bZIPinf突变型组患者年龄更小(54±64岁,P=0.010),初治AML更为常见(P=0.001),骨髓原始细胞比例更高(68.0%±36.3%,P=0.001)。此外,CEBPA-bZIPinf突变型组24例患者和CEBPA-其他突变型组19例患者接受了化疗。CEBPA-bZIPinf突变型组的一疗程完全缓解(CR)率显著高于CEBPA-其他突变型组(87.5%±47.4%,P=0.010)和CEBPA野生型组(87.5%±50.3%,P=0.002)。中位随访11个月后,CEBPA-bZIPinf突变型组的中位总生存期显著长于CEBPA野生型组(未达到vs22.1个月,P=0.012)。CEBPA-bZIPinf突变型AML是一种独特的临床实体,诊断年龄较轻,对化疗反应较好,预后较好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b389/11310808/93758d049e15/cjh-45-06-556-g001.jpg

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