Long-term follow-up of cytogenetically normal CEBPA-mutated AML.
Long-term follow-up of cytogenetically normal CEBPA-mutated AML.
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DOI：
10.1186/s13045-014-0055-7
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发表时间：
2014-09-10
影响因子：
28.5
通讯作者：
Spiekermann K
中科院分区：
文献类型：
--
作者：
Pastore F;Kling D;Hoster E;Dufour A;Konstandin NP;Schneider S;Sauerland MC;Berdel WE;Buechner T;Woermann B;Braess J;Hiddemann W;Spiekermann K
本研究的目的是分析CEBPA突变的AML患者的长期生存。我们研究了88例AML患者，他们的中位年龄为61岁，分别是(1)细胞遗传学正常的AML (CN-AML),(2)单等位基因（moCEBPA）或双等位基因（biCEBPA） CEBPA突变，以及(3)强化诱导治疗。88例患者中有60例既往随访时间较短。中位随访时间为9.8年（95% CI: 9.4-10.1年），而我们之前的分析为3.2年和5.2年。与moCEBPA患者相比，biCEBPA突变患者的生存时间明显更长（中位总生存期（OS） 9.6年对1.7年，p = 0.008）。≤60岁且biCEBPA突变的患者预后良好，10年OS率为81%。bi和mocebpa突变组的早期死亡率（d60）均较低，分别为7%和9%。biCEBPA和mocebpa突变患者的完全缓解（CR）率分别为82%和70% （p = 0.17）。与mocebpa突变患者相比，bicebpa突变患者的无复发生存期（RFS）更长（中位RFS为9.4年vs. 1.5年，p = 0.021），累积复发发生率（CIR）更低。这些OS和RFS的差异在校正了已知的临床和分子预后因素后得到证实。在这项长期观察中，我们证实了与mocebpa突变的CN-AML相比，biCEBPA突变患者的预后更好。年轻患者发生OS的高概率（81%）有助于指导缓解后治疗的强度。本文的在线版本（doi:10.1186/s13045-014-0055-7）包含补充材料，仅供授权用户使用。
The aim of this study was to analyze the long-term survival of AML patients with CEBPA mutations. We investigated 88 AML patients with a median age of 61 years and (1) cytogenetically normal AML (CN-AML), (2) monoallelic (moCEBPA) or biallelic (biCEBPA) CEBPA mutation, and (3) intensive induction treatment. 60/88 patients have been described previously with a shorter follow-up. Median follow-up time was 9.8 years (95% CI: 9.4-10.1 years) compared to 3.2 and 5.2 years in our former analyses. Patients with biCEBPA mutations survived significantly longer compared to those with moCEBPA (median overall survival (OS) 9.6 years vs. 1.7 years, p = 0.008). Patients ≤ 60 years and biCEBPA mutations showed a favorable prognosis with a 10-year OS rate of 81%. Both, bi- and moCEBPA-mutated groups had a low early death (d60) rate of 7% and 9%, respectively. Complete remission (CR) rates for biCEBPA- and moCEBPA-mutated patients were 82% vs. 70% (p = 0.17). biCEBPA-mutated patients showed a longer relapse free survival (RFS) (median RFS 9.4 years vs. 1.5 years, p = 0.021) and a lower cumulative incidence of relapse (CIR) compared to moCEBPA-mutated patients. These differences in OS and RFS were confirmed after adjustment for known clinical and molecular prognostic factors. In this long-term observation we confirmed the favorable prognostic outcome of patients with biCEBPA mutations compared to moCEBPA-mutated CN-AML. The high probability of OS (81%) in younger patients is helpful to guide intensity of postremission therapy. The online version of this article (doi:10.1186/s13045-014-0055-7) contains supplementary material, which is available to authorized users.
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影响因子：
158.5
作者：
Falini, B;Mecucci, C;Martelli, MF
通讯作者：
Martelli, MF
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