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5- 氮杂胞苷治疗骨髓增生异常综合征患者中 17 号染色体异常的预后意义:来自希腊 5- 氮杂胞苷登记处的结果。

The prognostic significance of chromosome 17 abnormalities in patients with myelodysplastic syndrome treated with 5-azacytidine: Results from the Hellenic 5-azacytidine registry.

机构信息

Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.

Institute of Nuclear and Radiological Sciences, National Center for Scientific Research Demokritos, Athens, Greece.

出版信息

Cancer Med. 2019 May;8(5):2056-2063. doi: 10.1002/cam4.2090. Epub 2019 Mar 21.

DOI:10.1002/cam4.2090
PMID:30897290
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6536924/
Abstract

In patients with myelodysplastic syndrome (MDS), the prognostic significance of chromosome 17 abnormalities has not yet been fully elucidated, except for isochromosome 17q that has been characterized as an intermediate risk abnormality in the Revised International Prognostic Scoring System (IPSS-R). To further characterize the prognostic significance of chromosome 17 abnormalities we analyzed the hematologic and prognostic characteristics of 548 adult patients with MDS treated with 5-azacytidine through the Hellenic 5-azacytidine registry and found 32 patients with a chromosome 17 abnormality (6 with i[17q], 15 with -17, 3 with add[17p] and the rest with other rarer abnormalities, mostly translocations). The presence of a chromosome 17 abnormality was correlated with poor prognostic features (high IPSS, IPSS-R, and WPSS scores) and a low overall survival rate (15.7 vs 36.4 months for patients without chromosome 17 abnormalities, Kaplan-Meier, Log Rank P < 0.00001), but these results were confounded by the fact that most (92.3%) of the cases with a chromosome 17 abnormality (with the exception of i(17q) that was found in all cases as an isolated abnormality) were found in the context of a complex karyotype. Nevertheless, one should not ignore the contribution of chromosome 17 abnormalities to the prognostic significance of a complex karyotype since 33.8% of complex karyotypes encompassed a chromosome 17 abnormality.

摘要

在骨髓增生异常综合征(MDS)患者中,除了 17 号染色体等臂异常(i[17q])被国际预后积分系统修订版(IPSS-R)定义为中危异常外,染色体 17 异常的预后意义尚未完全阐明。为了进一步阐明染色体 17 异常的预后意义,我们分析了通过希腊 5-氮杂胞苷注册研究接受 5-氮杂胞苷治疗的 548 例成人 MDS 患者的血液学和预后特征,发现 32 例患者存在染色体 17 异常(6 例 i[17q],15 例-17,3 例 add[17p],其余为其他更罕见的异常,主要为易位)。染色体 17 异常与预后不良特征(高 IPSS、IPSS-R 和 WPSS 评分)和总生存率低相关(无染色体 17 异常患者为 15.7 个月,有染色体 17 异常患者为 36.4 个月,Kaplan-Meier,Log Rank P<0.00001),但这些结果受到大多数染色体 17 异常(除了 i(17q),它作为一种孤立异常存在于所有病例中)存在于复杂核型背景这一事实的干扰。然而,人们不应忽视染色体 17 异常对复杂核型预后意义的贡献,因为 33.8%的复杂核型包含染色体 17 异常。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dfa9/6536924/a4dce13d0198/CAM4-8-2056-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dfa9/6536924/a4dce13d0198/CAM4-8-2056-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dfa9/6536924/a4dce13d0198/CAM4-8-2056-g001.jpg

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