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单倍体和复杂核型作为接受阿扎胞苷治疗的国际预后评分系统高危骨髓增生异常综合征患者的预后参数。

Monosomal and complex karyotypes as prognostic parameters in patients with International Prognostic Scoring System higher risk myelodysplastic syndrome treated with azacitidine.

作者信息

Hwang Kyung-Lim, Song Moo-Kon, Shin Ho-Jin, Na Hae-Jung, Shin Dong-Hun, Kim Joong-Keun, Moon Joon-Ho, Ahn Jae-Sook, Song Ik-Chan, Hong Junshik, Lee Gyeong-Won, Chung Joo-Seop

机构信息

Department of Hematology-Oncology, Pusan National University Hospital, Busan, Korea.

Department of Hematology, Kyungpook National University Hospital, Daegu, Korea.

出版信息

Blood Res. 2014 Dec;49(4):234-40. doi: 10.5045/br.2014.49.4.234. Epub 2014 Dec 23.

DOI:10.5045/br.2014.49.4.234
PMID:25548756
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4278004/
Abstract

BACKGROUND

Azacitidine (AZA) is standard care for patients with myelodysplastic syndrome (MDS) who have not had allogeneic stem cell transplantation. Chromosomal abnormalities (CA) including complex karyotype (CK) or monosomal karyotype (MK) are associated with clinical outcome in patients with MDS.

METHODS

We investigated which prognostic factors including CAs would predict clinical outcomes in patients with International Prognostic Scoring System (IPSS) higher risk MDS treated with AZA, retrospectively. CK was defined as the presence of three or more numerical or structural CAs. MK was defined as the presence of two or more distinct autosomal monosomies or single autosomal monosomy with at least one additional structural CA.

RESULTS

A total of 243 patients who treated with AZA, were enrolled. CK was present in 124 patients and MK was present in 90 patients. Bone marrow blasts ≥15% and CK were associated with poorer response (P=0.038, P=0.007) and overall survival (OS) (P<0.001, P<0.001) independently. Although MK in CK group was not associated with prognosis, non-MK status in non-CK group reflected favorable OS (P=0.005). The group including >3 CAs was associated with poorer OS (group including <3 CAs vs. only three CAs, P=0.001; group with >3 CAs vs. only three CAs, P=0.001).

CONCLUSION

CK was an important prognostic parameter associated with worse outcome. MK may predict poor survival in only non-CK status. The higher number of CAs was associated with poorer survival.

摘要

背景

阿扎胞苷(AZA)是未进行异基因干细胞移植的骨髓增生异常综合征(MDS)患者的标准治疗方法。包括复杂核型(CK)或单倍体核型(MK)在内的染色体异常(CA)与MDS患者的临床结局相关。

方法

我们回顾性研究了哪些预后因素(包括CA)可预测接受AZA治疗的国际预后评分系统(IPSS)高危MDS患者的临床结局。CK定义为存在三个或更多数量或结构上的CA。MK定义为存在两个或更多不同的常染色体单体或单个常染色体单体以及至少一个额外的结构CA。

结果

共有243例接受AZA治疗的患者入组。124例患者存在CK,90例患者存在MK。骨髓原始细胞≥15%和CK分别独立与较差的缓解(P = 0.038,P = 0.007)及总生存期(OS)(P < 0.001,P < 0.001)相关。虽然CK组中的MK与预后无关,但非CK组中的非MK状态提示OS良好(P = 0.005)。包括>3个CA的组与较差的OS相关(包括<3个CA的组与仅三个CA的组相比,P = 0.001;>3个CA的组与仅三个CA的组相比,P = 0.001)。

结论

CK是与较差结局相关的重要预后参数。MK可能仅在非CK状态下预测较差的生存期。CA数量越多与生存期越差相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b47/4278004/5a9959ddb891/br-49-234-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b47/4278004/f147a9c578a9/br-49-234-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b47/4278004/5a9959ddb891/br-49-234-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b47/4278004/f147a9c578a9/br-49-234-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b47/4278004/5a9959ddb891/br-49-234-g002.jpg

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Monosomal karyotype in MDS: explaining the poor prognosis?MDS 中的单体核型:解释不良预后的原因?
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Complex, not monosomal, karyotype is the cytogenetic marker of poorest prognosis in patients with primary myelodysplastic syndrome.复杂核型而非单倍体核型是原发性骨髓增生异常综合征患者预后最差的细胞遗传学标志物。
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