Myelodysplastic syndrome with chromosome 5 abnormalities: a nationwide survey in Japan
Myelodysplastic syndrome with chromosome 5 abnormalities: a nationwide survey in Japan
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DOI：
10.1038/leu.2008.199
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发表时间：
2008-10-01
期刊：
影响因子：
11.4
通讯作者：
Ozawa, K.
中科院分区：
文献类型：
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作者：
Tasaka, T.;Tohyama, K.;Ozawa, K.
5号染色体异常，5号染色体长臂缺失（del（5 q））或5号单体（-5），出现在约10%的骨髓增生异常综合征（MDS）中，作为唯一的细胞遗传学异常或复杂核型的一部分，并对MDS具有独特的临床意义。然而，5号染色体异常的MDS患者的预后因素尚未确定。在这项研究中，分析了183例日本MDS患者的5号染色体异常。MDS患者中del（5 q）和5 q综合征的估计发生率分别为8.4%和1.3%。与del（5 q）患者相比，-5患者的总生存期（OS）和无白血病生存期（LFS）显著缩短。在del（5 q）患者中，增加7号单体染色体或复杂核型并伴有3个以上异常与OS缩短显著相关。采用国际预后评分系统（IPSS）将del（5 q）患者的LFS分为低/中（Int）-1和Int-2/高2个危险组。根据世界卫生组织预后评分系统（WPSS）将LFS分为极低/低/Int和高/极高两组，WPSS较IPSS更能预测del（5 q）患者的预后，结合其他细胞遗传学资料，WPSS可为del（5 q）MDS患者的临床决策提供参考。
Chromosome 5 abnormalities, deletion of the long arm of chromosome 5 (del(5q)) or monosomy 5 (-5), arise in about 10% of myelodysplastic syndromes (MDS), either as the sole cytogenetic abnormality or as part of complicated karyotype, and has distinct clinical implications for MDS. However, the prognostic factors of MDS patients with chromosome 5 abnormalities are not determined yet. In this study, 183 Japanese MDS patients with chromosome 5 abnormalities were analyzed. Estimated incidence of del(5q) and 5q-syndrome among MDS patients was 8.4 and 1.3%, respectively. Significant shorter overall survival (OS) and leukemia-free survival (LFS) were observed in -5 patients than del(5q) patients. Among del(5q) patients, addition of monosomy 7 or complex karyotype with more than three abnormalities were significantly related to shorter OS.LFS of del(5q) patients was divided into two risk groups by international prognostic scoring system (IPSS): low/intermediate (Int)-1 and Int-2/high groups. LFS sorted by World Health Organization classification-based prognostic scoring system (WPSS) was also divided into two groups: very low/low/Int and high/very high, and WPSS was able to predict the outcome of del(5q) patients more clearly than IPSS.Together with additional cytogenetic data, WPSS might be useful for clinical decision making in MDS patients with del(5q).