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231 例单中心骨髓增生异常综合征患者的细胞遗传学特征及预后分析。

Cytogenetic characteristics and prognosis analysis in 231 myelodysplastic syndrome patients from a single institution.

机构信息

Department of Laboratory Medicine, The Catholic University of Korea, Seoul, Republic of Korea.

出版信息

Leuk Res. 2011 Jun;35(6):735-40. doi: 10.1016/j.leukres.2010.11.009. Epub 2010 Dec 13.

DOI:10.1016/j.leukres.2010.11.009
PMID:21146871
Abstract

We analyzed the clinical and hematologic data of 231 patients diagnosed with de novo myelodysplastic syndrome (MDS), identified cytogenetic characteristics, and evaluated the significance of prognostic systems. The median age was 51 years and the distribution of MDS subtypes demonstrated a markedly low incidence of MDS with deletion 5q (0.9%). The proportions of World Health Organization (WHO) categories differed according to patient age group. Refractory anemia with excess blasts-2 demonstrated the most significant trend toward increased frequency with advancing age. The incidence of abnormal karyotypes in our study was comparable to a previous study (50.2%), although with different patterns. The most frequent cytogenetic abnormality was +8 (34.5% of patients with abnormality), followed by 1q+ (17.2%), 5q- (15.5%), and 20q- (12.9%). Majority of +8, 1q+, -5/5q- and -7/7q- cases combined with additional cytogenetic abnormalities (60.0%, 75.0%, 88.5% and 100%, respectively). The median survival time was 49.5 months and 13.8% patients developed acute leukemia. WHO Prognostic Scoring System (WPSS) and age group were significant factors associated with overall survival. Otherwise, International Prognostic Scoring System was not included in the model. These results demonstrated the different cytogenetic features in Korean MDS patients compared to those of Western country. In addition, WPSS and age group are applicable to our patients as an effective and reliable prognostic model.

摘要

我们分析了 231 例初诊骨髓增生异常综合征(MDS)患者的临床和血液学数据,确定了细胞遗传学特征,并评估了预后系统的意义。中位年龄为 51 岁,MDS 亚型分布显示 5q 缺失型 MDS 的发生率明显较低(0.9%)。根据患者年龄组,世界卫生组织(WHO)类别比例不同。难治性贫血伴原始细胞增多-2 随着年龄的增长,频率呈显著增加趋势。我们的研究中异常核型的发生率与以前的研究相似(50.2%),尽管模式不同。最常见的细胞遗传学异常是+8(34.5%的异常患者),其次是 1q+(17.2%)、5q-(15.5%)和 20q-(12.9%)。大多数+8、1q+、-5/5q-和-7/7q-病例与其他细胞遗传学异常相结合(分别为 60.0%、75.0%、88.5%和 100%)。中位生存时间为 49.5 个月,13.8%的患者发生急性白血病。WHO 预后评分系统(WPSS)和年龄组是与总生存相关的显著因素。国际预后评分系统未包含在模型中。这些结果表明,与西方国家的 MDS 患者相比,韩国 MDS 患者的细胞遗传学特征不同。此外,WPSS 和年龄组可作为我们患者的有效和可靠预后模型。

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