Differing clinical features between Japanese and Caucasian patients with myelodysplastic syndromes: Analysis from the International Working Group for Prognosis of MDS
Differing clinical features between Japanese and Caucasian patients with myelodysplastic syndromes: Analysis from the International Working Group for Prognosis of MDS
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DOI：
10.1016/j.leukres.2018.08.022
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发表时间：
2018-10-01
影响因子：
2.7
通讯作者：
Greenberg, Peter L.
中科院分区：
文献类型：
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作者：
Miyazaki, Yasushi;Tuechler, Heinz;Greenberg, Peter L.
骨髓增生异常综合征(MDS)的临床特征可能受多种因素的影响，如疾病内在因素(如形态、细胞遗传学、分子)、外在因素(如管理、环境)和种族。之前的几项研究表明，亚洲和欧洲/美国国家之间存在这种差异。在这项研究中，为了阐明日本人(JPN)和高加索人(CAUC)中原发未经治疗的MDS的潜在差异，我们分析了国际MDS预后工作组收集的大型国际数据库中的数据(分别为300名和5838名患者)。JPN MDS较年轻，细胞减少较严重，细胞遗传学差异较大：del(5q)较少，+1/+1q、-1/del(1p)、der(1；7)、-9/del(9q)、del(16q)和del(20q)较多。虽然急性髓系白血病转化的时间没有差异，但即使在调整了年龄和FAB亚型后，JPN的存活率也显著提高，尤其是在低预后风险类别，但在高预后风险类别中并不明显。某些临床因素(细胞减少、原始细胞百分率、细胞遗传学风险)对两组患者的存活率和转化为白血病的时间有不同的影响。虽然不能排除可能的混杂因素(例如，环境、饮食和获得护理的机会)，但我们的结果表明，在JPN和CAUC患者的MDS存活率方面存在临床相关的种族差异。IPSS-R在CAUC和JP患者中的良好表现表明，其共同的风险模型适用于CAUC和JP。
Clinical features of myelodysplastic syndromes (MDS) could be influenced by many factors, such as disease intrinsic factors (e.g., morphologic, cytogenetic, molecular), extrinsic factors (e.g, management, environment), and ethnicity. Several previous studies have suggested such differences between Asian and European/USA countries. In this study, to elucidate potential differences in primary untreated MDS between Japanese (JPN) and Caucasians (CAUC), we analyzed the data from a large international database collected by the International Working Group for Prognosis of MDS (300 and 5838 patients, respectively). JPN MDS were significantly younger with more severe cytopenias, and cytogenetic differences: less del(5q) and more +1/+1q, -1/del(1p), der(1;7), -9/del(9q), del(16q), and del(20q). Although differences in time to acute myeloid leukemia transformation did not occur, a significantly better survival in JPN was demonstrated, even after the adjustment for age and FAB subtypes, especially in lower, but not in higher prognostic risk categories. Certain clinical factors (cytopenias, blast percentage, cytogenetic risk) had different impact on survival and time to transformation to leukemia between the two groups. Although possible confounding events (e.g., environment, diet, and access to care) could not be excluded, our results indicated the existence of clinically relevant ethnic differences regarding survival in MDS between JPN and CAUC patients. The good performance of the IPSS-R in both CAUC and JP patients underlines that its common risk model is adequate for CAUC and JP.