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伴有 IPSS 定义的低危骨髓增生异常综合征患者细胞遗传学异常的获得与不良预后和向急性髓系白血病转化相关。

Acquisition of cytogenetic abnormalities in patients with IPSS defined lower-risk myelodysplastic syndrome is associated with poor prognosis and transformation to acute myelogenous leukemia.

机构信息

Department of Leukemia, the University of Texas, MD Anderson Cancer Center, Houston, Texas.

出版信息

Am J Hematol. 2013 Oct;88(10):831-7. doi: 10.1002/ajh.23513. Epub 2013 Jul 23.

DOI:10.1002/ajh.23513
PMID:23760779
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3923606/
Abstract

We hypothesized that the dynamic acquisition of cytogenetic abnormalities (ACA) during the follow up of myelodysplastic syndromes (MDS) could be associated with poor prognosis. We conducted a retrospective analysis of 365 patients with IPSS low or intermediate-1 risk MDS who had at least two consecutive cytogenetic analyses during the follow up. Acquisition of cytogenetic abnormalities was detected in 107 patients (29%). The most frequent alteration involved chromosome 7 in 21% of ACA cases. Median transformation-free and overall survival for patients with and without ACA were 13 vs. 52 months (P = 0.01) and 17 vs. 62 months (P = 0.01), respectively. By fitting ACA as a time-dependent covariate, multivariate Cox regression analysis showed that patients with ACA had increased risk of transformation (HR = 1.40; P = 0.03) or death (HR = 1.45; P = 0.02). Notably, female patients with therapy-related MDS (t-MDS) had an increased risk of developing ACA (OR = 5.26; P < 0.0001), although subgroup analysis showed that prognostic impact of ACA was not evident in t-MDS. In conclusion, ACA occurs in close to one third of patients with IPSS defined lower risk MDS, more common among patients with t-MDS, but has a significant prognostic impact on de novo MDS.

摘要

我们假设骨髓增生异常综合征(MDS)患者在随访过程中细胞遗传学异常(ACA)的动态获得可能与预后不良有关。我们对至少有两次连续细胞遗传学分析的 365 例 IPSS 低危或中危-1 风险 MDS 患者进行了回顾性分析。在 107 例患者(29%)中检测到细胞遗传学异常的获得。最常见的改变涉及 ACA 病例中 21%的染色体 7。有和没有 ACA 的患者无转化和总生存的中位数分别为 13 个月与 52 个月(P = 0.01)和 17 个月与 62 个月(P = 0.01)。通过将 ACA 拟合为一个时间依赖性协变量,多变量 Cox 回归分析表明,有 ACA 的患者转化(HR = 1.40;P = 0.03)或死亡(HR = 1.45;P = 0.02)的风险增加。值得注意的是,治疗相关 MDS(t-MDS)的女性患者发生 ACA 的风险增加(OR = 5.26;P < 0.0001),尽管亚组分析显示 ACA 在 t-MDS 中对预后的影响不明显。总之,在 IPSS 定义的低危 MDS 患者中,近三分之一的患者出现 ACA,在 t-MDS 患者中更为常见,但对初发性 MDS 有显著的预后影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5057/3923606/6b945915e249/nihms549015f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5057/3923606/519d52448589/nihms549015f1a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5057/3923606/3d4f1eb4e4e0/nihms549015f2a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5057/3923606/6b945915e249/nihms549015f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5057/3923606/519d52448589/nihms549015f1a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5057/3923606/3d4f1eb4e4e0/nihms549015f2a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5057/3923606/6b945915e249/nihms549015f3.jpg

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